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I put together a few things I've collected that may help to connect some of

the various theories. Remember that the Etiology of autism info from CPEA

mentions immune abnormalities and genetic hotspots, especially on Chromosome

7. There are also similar related findings in other disorders. I guess

there are alot of roads that can lead to a similar destination. To make it

more interesting/confusing, there is even something on ABO bloodtype. I'm

AB+

Source: University Of Southern California (http://www.usc.edu/)

Date: Posted 3/20/2002

USC Researchers Define Role Of Protein, Discover Cause Of Chromosome Damage

Pinpointing oxygen as the cause of routine chromosome damage and defining

the role of a key protein in the repair of that damage are the subjects of

two recently published papers from the laboratory of USC/Norris

Comprehensive Cancer Center pathologist Lieber, the Rita and

Polusky Chair in Basic Cancer Research at the Keck School of Medicine.

The first paper was published in the March 5, 2002, issue of Current

Biology; the other is slated for the March 22 issue of Cell, but was posted

on the journal's Web site on March 1 as part of their " immediate early

publication process. "

The Cell paper, which will reportedly be on the cover of the March 22 issue,

reveals that a protein previously linked to a devastating form of

immunodeficiency plays a key role in a pathway by which nuclear DNA is

repaired—the same system, in fact, which the immune system uses to create

antibodies.

About 15 percent of the cases of human severe combined immunodeficiency

syndrome (known colloquially as the " bubble boy " disease) are caused by the

mutation of a specific gene and its protein product. In April of 2001, a

team of French researchers tracked down that gene, and named it and its

product Artemis (after the Greek goddess for the protection of children);

they had no idea at all, however, what kind of protein it was, nor what its

function might be.

Enter Lieber and graduate student Yunmei Ma. Lieber, Ma and their colleagues

from the University of Ulm in Germany, conclusively demonstrated that

Artemis is a key protein in the repair of double-stranded DNA breaks, a

process called NHEJ (non-homologous DNA end joining). In the NHEJ pathway,

explained Lieber, the ends of the broken DNA strands are trimmed and

rejoined to one another. " What Artemis does is trim away the damaged parts

of the DNA so that the strands can be joined, " said Lieber.

Artemis and the NHEJ pathway are so essential, Lieber continued, that mice

lacking NHEJ usually die at birth-and those that don't generally lack an

immune system entirely and experience accelerated aging. And, as the

previous studies have shown, humans with a defective Artemis protein also

wind up without any immune defense to speak of. That, says Lieber, is

because the immune system creates its defenses by cutting and then rejoining

bits of nuclear DNA (the rejoining relies on NHEJ). Without Artemis, the

cells can't create the antibodies necessary to go after the myriad

pathogenic invaders we regularly encounter.

Of course, being unable to cut and splice DNA can sometimes actually be of

benefit. " What we're going to do next, " said Ma, the paper’s first author,

" is try to screen for drugs that inhibit Artemis, because this might be

useful from a cancer therapy standpoint. If we could just give a pulse of

drug inhibitor for a while, we might be able to focus the effects of

radiation therapy, for instance, by not allowing the cancer cells to repair

themselves after being hit with the radiation. "

Still, noted Lieber, for normal cells, Artemis and the NHEJ pathway are

absolutely critical for survival. And that is because of how exquisitely

vulnerable our cells are to DNA damage in the first place. Indeed, he said,

all you have to do is take some cells out of the organism in which they live

and look at them under a microscope, and you'll find that 5 to 10 percent of

them will have at least one broken chromosome.

Normally, of course, the NHEJ pathway works to fix those breaks. But the

NHEJ pathway doesn't always function at full capacity. Indeed, a paper

published by Lieber, M.D./Ph.D. student Zarir E. Karanjawala, and Norris

Cancer Center researcher Chih-Lin Hsieh in 1999 found that in cells where

the NHEJ pathway is disabled or missing, the number of cells with at least

one chromosome break goes shooting up to 60 percent.

What causes all this breakage? In the March 5 issue of Current Biology,

Karanjawala, Lieber, and colleagues say it's the most ubiquitous of sources:

oxygen.

Originally, said Karanjawala, they had wondered if the damage might be

coming from some environmental source, perhaps from background radiation.

But when they began to look more closely, said Karanjawala, they found it

was in the very air we breathe. " It's coming from the oxygen, " Karanjawala

explained. " We found that if you vary the oxygen levels in which cells are

grown, the breakage levels of the chromosomes vary as well––the higher the

oxygen level, the more breakage you'll see. "

The oxygen causes its damage, Lieber said, through oxidative free

radicals—highly reactive atoms with an unpaired electron that can rip

through our cells " like a bullet. "

" Our bodies are being riddled with these bullets every day, " explained

Lieber, " whether we like it or not. And the sorts of double-strand DNA

breaks we were looking at are hard to repair. Even if you put the two ends

together the best you can, you usually lose a couple of nucleotides along

the way. And so every time we get an oxidative free radical hit, which

happens several times per day per cell, we lose a little info. Every time it

hits your DNA, you wind up with a little less genetic information than you

had when you started the day. "

The solution? ly, said Lieber, there may be none. " Oxygen—can't live

with it, can't live without it, " he commented. " We need it to survive, but

ultimately, it's also probably what kills us. "

--------------------------------------------------------------------------------

Note: This story has been adapted from a news release issued by University

Of Southern California for journalists and other members of the public. If

you wish to quote from any part of this story, please credit University Of

Southern California as the original source. You may also wish to include the

following link in any citation:

http://www.sciencedaily.com/releases/2002/03/020313075022.htm

----------------------------------------------------------------------

Source: University Of Michigan Health System (http://www.med.umich.edu/)

Date: Posted 5/15/2002

Parasite Or Partner? Study Suggests New Role For Junk DNA

ANN ARBOR, Mich. – Junk DNA is the Rodney Dangerfield of the genetics world.

It makes up nearly half of all human DNA, but many scientists dismiss it as

useless gibberish. A new study published online today from the June 2002

issue of Nature Genetics, however, suggests that segments of junk DNA called

LINE-1 elements deserve more respect.

Conducted by scientists from the University of Michigan Medical School and

Louisiana State University, the study is the first to show in mammalian

cells that some human LINE-1, or L1, elements can jump to chromosomes with

broken strands of DNA, slip into the break and repair the damage.

“Transposable L1 elements make up 17 percent of our DNA, but very little is

known about them,” says V. Moran, Ph.D., an assistant professor of

human genetics and internal medicine in the U-M Medical School, who

developed the first assay to identify mobile L1s in the human and mouse

genomes. “Until now, everyone thought L1s were just intracellular parasites

in our DNA – leftovers from the distant evolutionary past. The big question

in the field is: Are they still there because we can’t get rid of them or do

they have a function?”

L1s “reproduce” by using RNA and a process called reverse transcription to

make complementary DNA copies of themselves, which can jump into other DNA

sequences. Normally, L1s use an enzyme called endonuclease to cut the

genetic DNA and create a space, so they can plug themselves into the genome.

“We knew about the endonuclease pathway,” says Tammy A. h, a U-M

graduate student in human genetics and first author of the paper. “But we

didn’t know there was another mechanism that didn’t require endonuclease, or

that L1s could jump into existing breaks in DNA.”

h tested human L1s’ ability to repair DNA breaks in several normal and

DNA-repair mutant cell lines derived from Chinese hamster ovary cells. Other

researchers had demonstrated the ability of human L1s to repair DNA breaks

in yeast cells, but h is the first to show the effect can occur in

mammalian cells.

Since DNA damage may lead to cell death unless it is repaired, the existence

of an alternate repair pathway could be a good thing for the host cell. The

question is, what’s in it for the L1?

“This study brings up the question of whether L1s are just taking advantage

of DNA breaks to plug themselves into these sites or are they are being used

by the host cell to mediate the repair,” says Moran. “From the L1s’ point of

view, this gives it an alternate way of integrating into the DNA.”

Because L1s are so ancient and because they sometimes carry segments of

genes with them when they jump to a new location, Moran believes they have

played an important role in human evolution by increasing genetic diversity.

He is one of only a few scientists to study L1s in the human genome.

“We have more transposable L1s in the human genome than any other species,

but we know the least about where and how they move in humans,” says Moran.

“We are here today either because of, or in spite of, L1s.”

In future research, Moran’s research team will examine whether it is

possible to direct L1s to repair specific breaks in DNA, whether L1s can be

used as vectors to deliver genetic material to specific DNA locations, and

the impact of an L1 insertion on genes.

U-M researchers in the study were supported by the M. Keck

Foundation and the National Institutes of Health. Gilbert, Ph.D., a

U-M post-doctoral fellow in human genetics, collaborated in the study. Other

collaborators included Mark A. Batzer, S. Myers, and Bethaney J.

from Louisiana State University; D. Stamato from Lankenau

Institute for Medical Research in Wynnewood, Penn.; and Guillermo E.

Taccioli from Boston University’s School of Medicine.

Editor's Note: The original news release can be found at

http://www.med.umich.edu/opm/newspage/2002/junkdna.htm

-----------------------------------------------------------------------

(MY NOTE-some of you may remember the research on Gulf War Illness where

there were findings of DNA in the blood and mention of this chromosomal

area)

THYROID AUTOANTIGEN, 70-KD; G22P1

Alternative titles; symbols

Ku ANTIGEN, 70-KD SUBUNIT; Ku70

LUPUS AUTOANTIGEN p70

THYROID-LUPUS AUTOANTIGEN; TLAA

Gene map locus 22q11-q13

The G22P1 gene encodes subunit p70 of the p70/p80 autoantigen. The p70/p80

autoantigen consists of 2 proteins of molecular mass of approximately 70,000

and 80,000 daltons that dimerize to form a 10 S DNA-binding complex. See

194364 for discussion of the gene encoding the p80 subunit. Exchange of

immunologic reagents showed that the p70/p80 autoantigen is identical to the

Ku antigen, the Ki antigen, and the 86- to 70-kD protein complex. The

p70/p80 complex binds to the ends of double-stranded DNA in a cell

cycle-dependent manner, being associated with chromosomes of interphase

cells, followed by complete dissociation from the condensing chromosomes in

early prophase. Both p70 and p80 contain phosphoserine residues. A role for

the antigen in DNA repair or transposition has been proposed.

Taccioli et al. (1994) showed through genetic and biochemical approaches

that the XRCC5 is the 80-kilodalton subunit of the Ku protein. Ku binds to

free double-stranded DNA ends and is the DNA-binding component of the

DNA-dependent protein kinase. Thus, the Ku protein is involved in DNA repair

and in V(D)J recombination, and the Ku-DNA-dependent protein kinase complex

may have a role in those same processes.

------------------------------------------------------------------------

EMBO J 2002 Apr 15;21(8):2038-44 Related Articles, Books, LinkOut

Specific interaction of IP(6) with human Ku70/80, the DNA-binding subunit of

DNA-PK.

Hanakahi LA, West SC.

Cancer Research UK, London Research Institute, Clare Hall Laboratories,

South Mimms, Hertfordshire EN6 3LD, UK Corresponding author e-mail:

stephen.west@...

In eukaryotic cells, DNA double-strand breaks can be repaired by

non-homologous end-joining, a process dependent upon Ku70/80, XRCC4 and DNA

ligase IV. In mammals, this process also requires DNA-PK(cs), the catalytic

subunit of the DNA-dependent protein kinase DNA-PK. Previously, inositol

hexakisphosphate (IP(6)) was shown to be bound by DNA-PK and to stimulate

DNA-PK-dependent end-joining in vitro. Here, we localize IP(6) binding to

the Ku70/80 subunits of DNA- PK, and show that DNA-PK(cs) alone exhibits no

detectable affinity for IP(6). Moreover, proteolysis mapping of Ku70/80 in

the presence and absence of IP(6) indicates that binding alters the

conformation of the Ku70/80 heterodimer. The yeast homologue of Ku70/80,

yKu70/80, fails to bind IP(6), indicating that the function of IP(6) in

non-homologous end-joining, like that of DNA-PK(cs), is unique to the

mammalian end-joining process.

PMID: 11953323 [PubMed - in process]

Mutagenesis 2002 May;17(3):193-200 Related Articles, Books, LinkOut

Environmental factors affecting transcription of the human L1

retrotransposon. I. Steroid hormone-like agents.

Morales JF, Snow ET, Murnane JP.

Radiation Oncology Research Laboratory, University of California-San

Francisco, 1855 Folsom Street, MCB 200, San Francisco, CA 94103, USA and

Centre for Cellular and Molecular Biology, School of Biological and Chemical

Sciences, Deakin University, 221 Burwood Highway, Burwood, 3125 ,

Australia.

The L1 retrotransposon has significantly shaped the structure of the human

genome. At least 30% of human genome sequence can be attributed to L1

reverse transcriptase activity. There are 10(5) copies of the human L1

retrotransposon, L1Hs, most of which are defective, although approximately

8-9x10(3) are full length. L1Hs elements transpose through an RNA

intermediate and transcription is thought to be the rate limiting step in

retrotransposition. Because transcription of retrotransposons in a variety

of organisms has been shown to respond to environmental stimuli, we

investigated the influence of various agents on transcription from two

different L1Hs promoters. The activity of the L1Hs promoters was analyzed by

transfecting L1Hs-expressing cell lines with plasmids containing the L1Hs

promoters fused to the LacZ reporter gene and monitoring expression with a

beta-galactosidase assay. Small increases in beta-galactosidase activity

were observed with both L1Hs promoters after treatment with serum,

testosterone, dihydrotestosterone and organochloride pesticides, indicating

that these agents can influence L1Hs transcription.

PMID: 11971989 [PubMed - as supplied by publisher]

J Mol Evol 1999 Jun;48(6):675-83 Related Articles, Books, LinkOut

Comparison between two human endogenous retrovirus (HERV)-rich regions

within the major histocompatibility complex.

Kulski JK, Gaudieri S, Inoko H, Dawkins RL.

Centre for Molecular Immunology and Instrumentation, University of Western

Australia, Faculty of Medicine and Dentistry, P.O. Box 5100, Canning Vale,

6155, Western Australia, Australia. jkulski@....

Sixteen human endogenous retrovirus (HERV) sequences were detected within

656 kb of genomic sequence obtained from the alpha- and beta-block of the

class I region of the major histocompatibility complex (MHC). The HERVs were

identified and characterized as family members of HERV-16 (11 copies),

HERV-L (1 copy), HERV-I (2 copies), HERV-K91 (1 copy), and HARLEQUIN (1

copy) by sequence comparison using CENSOR or Repeat Masker, BLAST searches,

and dot plots. The 11 copies of HERV-16 arose as products of duplication of

genomic segments containing HLA class I (HLAcI) and PERB11 (MIC) genes inter

alia, whereas the other five HERVs arose after duplication probably as a

consequence of single insertion events or translocations. HERV-L and HERV-I

are located between the duplicated genes PERB11.2 (MICB) and PERB11.1

(MICA), and HLA-B and HLA-C, respectively, whereas HERV-K91 and HARLEQUIN

are located telomeric of HLA-C. A highly fragmented copy of HERV-I was also

found telomeric of PERB11. 4. Structural analysis of open reading frames

(ORFs) revealed the absence of intact coding sequence within the putative

gag, pol, and env gene regions of all the HERVs with the exception of

HERV-K91, which had two large ORFs within the region of the putative

protease and pol genes. In addition, the 5'-LTR of HERV-L contained a 2.5-kb

element that was AT-rich and large ORFs with putative amino acid sequences

rich in tyrosines and isoleucines. HERV-I, HARLEQUIN, and at least four

copies of HERV-16 appear to have been receptors for the insertion of other

retrotransposons including Alu elements and fragments of L1 and THE1.

Examination of flanking sequences suggests that HERV-I and HERV-L had

occurred by insertion into ancient L1 fragments. This study has revealed

that the alpha- and beta-block region within the MHC is rich in HERV

sequences occurring at a much higher ratio (10 to 1) than normally observed

in the human genome. These HERV sequences will therefore enhance further

studies on disease associations and differences between human haplotypes and

primates and their role in the evolution of class I genes in the MHC.

PMID: 10229571 [PubMed - indexed for MEDLINE]

Cell Mol Biol (Noisy-le-grand) 2002 Mar;48(2):213-7 Related Articles, Books,

LinkOut

The HERV-W/7q family in the human genome. Potential for protein expression

and gene regulation.

Alliel PM, Perin JP, Goudou D, Bitoun M, B, Rieger F.

INSERM U-488, Batiment Pincus, Le Kremlin-Bicetre, France.

alliel@...

A new family of human endogenous retroviruses has recently been discovered.

The best known example of a full length member of this family, HERV-W/7q, is

located on chromosome 7. HERV-W/7q is characterized by a long open reading

frame within its env gene which is expressed in various tissues, and mainly

in placenta, as a protein that we called enverin. A search for new

retroviral sequences related to the HERV-W/7q family allowed the

characterization of such elements in chromosome 6. A novel full length HERV

with an env gene of the HERV-W/7q type, potentially encoding a truncated

form of enverin has been identified on chromosome 10. The distribution of

HERV-W/7q related sequences close to or within genes offers the possibility

that the expression of these genes may be regulated by their companion

retroviral sequences.

PMID: 11990458 [PubMed - in process]

Neurovirol 2000 May;6 Suppl 2:S67-75 Related Articles, Books, LinkOut

Particle-associated retroviral RNA and tandem RGH/HERV-W copies on human

chromosome 7q: possible components of a 'chain-reaction' triggered by

infectious agents in multiple sclerosis?

Perron H, Perin JP, Rieger F, Alliel PM.

BioMerieux STELHYS, Chemin de l'Orme, 69280 Marcy l'Etoile, France.

Different groups have observed retrovirus particle (RVP) production in cell

cultures from patients with multiple sclerosis (MS). This in vitro

production appeared relatively specific for MS versus healthy controls, but

was likely to be enhanced or activated by infectious triggers such as

Herpesviruses (e.g. HSV, EBV). Independent molecular analysis of retroviral

RNA associated with RVP revealed two different genetic families of

endogenous retroviral elements (HERV): MSRV/HERV-W and RGH/HERV-H.

Interestingly, these sequences were detected by mutually exclusive primers

in RT - PCR amplifications. Surprisingly, these two HERV families both

contain an ancestral proviral copy inserted in chromosome 7q21-22 region at

about 1 kb of distance of each other. Another HERV-W proviral sequence is

located within a T-cell alpha-delta receptor (TCR) gene in chromosome

14q11.2 region. Interestingly, these two regions correspond to genetic loci

previously identified as potentially associated with 'multigenic'

susceptibility to MS and TCR alpha chain genetic determinants have been

reported to be statistically associated with MS. A plausible role for

infectious agents triggering a co-activation of the chromosome 7q HERV

tandem (replicative retrovirus and/or other virus and/or intracellular

bacteria) and, eventually, other HERV copies, is discussed. The role of

particular HERV polymorphism and the production of pathogenic molecules

(gliotoxin and superantigen) possibly associated with retroviral expression

are also evoked. An integrative concept of pathogenic 'chain-reaction' in MS

involving several step-specific pathogenic 'agents' and 'products' somewhat

interacting with particular genetic elements would federate most partial

data obtained on MS, including retroviral expression.

Publication Types:

Review

Review Literature

PMID: 10871789 [PubMed - indexed for MEDLINE]

Int J Mol Med 2002 Mar;9(3):207-12 Related Articles, Books, LinkOut

Schizophrenia: a genetic perspective (review).

Shastry BS.

Department of Biological Sciences, Oakland University, Rochester, MI 48309,

USA. Barkur@...

Schizophrenia is a complex neurodevelopmental disorder characterized by

mental dysfunction across multiple domains of the brain. It affects 1% of

world's general population and the nature of neurobiological lesions in the

schizophrenic brain are not known. Although the exact etiology of the

disorder is not understood, twin, family and adoption studies have provided

consistent evidence that genetic factors play a major role in the

pathogenesis. A genome-wide genetic linkage screen identified loci on

chromosomes 1, 2, 4, 5, 6, 7, 8, 9, 10, 11, 13, 15, 18, 22 and the X with

positive lod scores, thus excluding a single major locus for schizophrenia.

Association studies have generated disappointing results in identifying the

susceptible DNA sequence variants and the anticipation hypothesis on

trinucleotide repeat expansion provided equivocal results or lack of

enthusiasm. Although there are no biological markers at present, the recent

finding that human endogenous retrovirus is activated in cerebrospinal fluid

as well as in the postmortem schizophrenic brain may change our

understanding of the etiopathogenesis of this disease. Meanwhile, treatment

with newly developed anti-psychotic drugs combined with educational and

cognitive rehabilitation procedure may help the patients to cope with the

illness.

Publication Types:

Review

Review, Tutorial

PMID: 11836624 [PubMed - indexed for MEDLINE]

Mol Psychiatry 2001 Nov;6(6):634-46 Related Articles, Books, LinkOut

Schizophrenia and viral infection during neurodevelopment: a focus on

mechanisms.

Pearce BD.

Department of Psychiatry and Behavioral Sciences, Emory University School of

Medicine, 1639 Pierce Dr WMB-4000, Atlanta, GA 30322, USA. bpearce@...

The task of defining schizophrenia pathogenesis has fascinated and

frustrated researchers for nearly a century. In recent years, unprecedented

advances from diverse fields of study have given credence to both viral and

developmental theories. This review considers possible mechanisms by which

viral and developmental processes may interact to engender schizophrenia.

Many of the current controversies in schizophrenia pathogenesis are reviewed

in light of the viral hypothesis, including: epidemiological findings and

the role of a genetic diathesis, phenotype heterogeneity, abnormalities in

excitatory and inhibitory neurotransmitter systems, anomalous cerebral

latereralization, and static vs progressive disease. The importance of

animal models in elucidating the impact of viral infections on developing

neurons is illustrated by recent studies in which neonatal rats are infected

with lymphocytic choriomeningitis virus in order to examine alterations in

hippocampal circuitry. Finally, consideration is given to a new hypothesis

that some cases of schizophrenia could be instigated by a viral infection

that disrupts developing inhibitory circuits, consequently unleashing

glutamatergic neurotransmission leading to selective excitotoxicity, and a

degenerative disease course.

Publication Types:

Review

Review Literature

PMID: 11673791 [PubMed - indexed for MEDLINE]

Blood 2002 Apr 1;99(7):2477-82 Related Articles, Books, LinkOut

Expression of ABO or related antigenic carbohydrates on viral envelopes

leads to neutralization in the presence of serum containing specific natural

antibodies and complement.

Preece AF, Strahan KM, Devitt J, Yamamoto F, Gustafsson K.

Molecular Immunology Unit, Institute of Child Health, University College

London, 30 Guilford Street, London WC1N 1EH, United Kingdom.

No definitive biologic function has been associated with the human ABO

histo-blood group polymorphism, or any other terminal carbohydrate

differences within or between closely related species. We have

experimentally addressed the question of whether viral particles can become

glycosylated as determined by the glycosylation (eg, ABO) status of the

producer cell and as a result be affected by human serum containing specific

natural antibodies (NAbs). Measles virus was produced in cells transfected

with cDNA encoding, either human A-transferase, B-transferase, an inactive

" O-transferase, " or a pig alpha1-3galactosyltransferase (alpha1-3GT)

synthesizing the Galalpha1-3Gal structure. The viruses were shown to carry

the same ABO structures as the cells; that is, A but not B if produced in

A-type cells, and B but not A if produced in B-type cells. Only O was

detected on the virus produced from O-type cells, whereas reduced amounts of

O appeared on the A- and B-type viral particles. In addition, the

Galalpha1-3Gal structure was transferred onto measles only when grown in

human cells expressing this structure. When subjected to human preimmune

sera, the A-type, the B-type, and the Galalpha1-3Gal viral particles were

partially neutralized in a complement-dependent manner. However, the O-type

or the Galalpha1-3Gal-negative viral particles were not neutralized. The

neutralization appeared to be mediated by specific NAb, as judged by

specific inhibition using synthetic A and Galalpha1-3Gal oligosaccharides.

Such viral glycosylation may thus partly explain why the ABO antigens and

other similar intraspecies as well as interspecies polymorphic carbohydrates

have evolved and been maintained over long evolutionary periods.

PMID: 11895782 [PubMed - indexed for MEDLINE]

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A consistency in the genetic studies of autism also reveal a clear

connection to the CYP 450 enzymes. Therefore the lutein theory as a

causal factor for autism identifies the source of the intensified free

radical production.

1p

Defects of chromosome 1p include: Cytochrome P450: CYP2J2 arachidonic

acid epoxygenase deficiency; CYP4B1 Parkinsonism. Porphyria cutania

tarda, light sensitive dermatitis and associated excresion of

uroporphyrin; hypertrichosis and hyperpigmentation also occur.

Waardenburg syndrome (WS) type 2B sensorinural hearing loss,

heterochromia irides, white forelock and early graying.

Oligodendrogliomas: 5

2q

Defects of chromosome 2q include: Cytochrome P450: Cyp27 (Sterol

27-hydroxylase): ?bile acid synthesis? and cerebrotendinous

xanthomatosis, cerebral cholesterinosis (CTX). Waardenburg syndrome

(WS): an inherited disorder often characterized by varying degrees of

hearing loss and changes in skin and hair pigmentation:

6q

Defects of chromosome 6q include: Congenital adrenal hyperplasia (CAH)

is an inherited recessive disorder of adrenal steroidogenesis caused

by mutations in the steroid 21-hydroxylase gene (CYP21) in more than

90% of affected patients. Retinitis Pigmentosa (RP) also found on

chromosomes 1p, 1q , 9q and 10p. 97, 8, 37 A clotting of the pigment

in the choroid structure of the eye. Retinitis pigmentosa is reported

in more than a coincidental number of families (parent or grandparent)

who have a child or grandchild with autism.85, 86, 91

?Immunodeficiency and IgA deficiency are related disorders,

susceptibility to which is determined by a gene(s) within or near the

MHC class III gene region on chromosome 6?. 108 IgA deficiency has

been reported in autism. 99 [NB: 6p and 10p: Refsum?s disease

(treatment with a chlorophyll free diet ? Eldjarn, L. et al 1966) is

characterized as a syndrome with RP and nerve deafness, ichthyosis,

cerebellar ataxia and polyneuritis.]

7q

Defects of chromosome 7q include: Cytochrome P450: Nitric Oxide

Synthase 3 (NOS3), endothelial nitric oxide synthase (ENOS). ?The

unexpected recognition of the substrate L-arginine at the H4B

(tetrahydrobiopterin) site indicates that this site is poised to

stabilize a positively charged pterin ring and suggests a model

involving a cationic pterin radical in the catalytic cycle? Raman et

al (1998); CYP3A4, CYP3A43 ?Xenobiotic? metabolism; CYP51, sterol

biosynthesis, cholesterol biosynthesis; CYP5, thromboxane A synthase,

bruises, nosebleeds, defective platelet aggregation. Speech Language

disorder (also occurring on 19q and 16q). Hemochromatosis (HFE3); 12,

13, 14, 36 a disorder due to deposition of hemo-siderin in the

parenchymal cells, causing tissue damage and dysfunction of the liver,

pancreas, heart, and pituitary. Other clinical signs include bronze

pigmentation of the skin, arthropathy, diabetes, cirrhosis,

hepatosplenomegaly, hypogonadism, and loss of body hair. Idiopathic

Hemochromatosis: an autosomal recessive disorder of iron metabolism

associated with a gene tightly linked to the A locus of the HLA

complex on chromosome 6.58

Hemochromatosis is also linked to Crystal Deposition Disease: ?CPPD

crystal deposition disease may be classified as hereditary (autosomal

dominant), sporadic (idiopathic), or associated with other metabolic

diseases, such as hyperparathyroidism, hemochromatosis,

hypothyroidism, or even gout itself. The acute pseudogout syndrome is

characterized by an abrupt onset of acute inflammatory gout-like

arthritis affecting one or more joints, most commonly the knee. Among

other clinical manifestations, about 50% of patients with CPPD deposit

disease present with progressive degeneration of multiple joints,

usually the knees, ankles, wrists, elbows, hips, or shoulders. The

initiating event for CPPD crystal deposition within joints is not well

understood. The pathogenesis of the acute inflammatory changes in the

joints are attributed to the uptake of CPPD crystals by

polymorphonuclear and mononuclear phagocytes, followed by release of

degradative enzymes, inflammatory mediators, and chemotactic factors,

very much as in gouty arthritis.? 73

In the autist, the typical presentation of these iron deposition

disorders is not found. Nevertheless, autists do often have problems

with iron metabolism, and even when anemic, supplementing with iron

can result in an unexpected drop in iron level. Likewise,

supplementing with Vitamin C has an unexpected negative outcome in

some autists.

[NB: Vitamin C Toxicity: ?Megadoses of vitamin C of 1000-2000 mg have

commonly been associated with gastrointestinal disturbances (nausea,

abdominal cramps and diarrhea). In general, megadoses of vitamin C

should be avoided in individuals with a history of renal stones due to

oxalate formation or hemochromatosis or other diseases related to

excessive iron accumulation. Excess vitamin C may predispose premature

infants to hemolytic anemia due to the fragility of their red blood

cells. In healthy individuals, it appears that megadoses of vitamin C

are well tolerated and not associated with any consistent adverse

effects.? 82, 77, 72 Vitamin C supplementation in excess of the RDA is

not always a good idea for people with ASD.]

13q

Defects associated with chromosome 13q include: ?s disease

(potentially resulting in jaundice), copper-transporting P-type

ATPases copper transport, Mutations of ATP7B. Currently NIH is funding

studies to clarify the association between ?s disease and

autism. In our experience we have encountered an autistic female who

presented with yellow eyes and also encountered many ASD females who

exhibited yellow eye color especially at or near the onset of

menstruation. Phylloid pattern of pigmentary disturbance (Ribeiro Noce

T et al). Hirschsprung disease type 2: Puffenberger et al. (1994)

presented evidence that Hirschsprung disease type 2 (HSCR2; 600155),

an apparently multigenic disorder, is due to mutation in the

endothelin-B receptor gene. EDNRB was a candidate gene because it

mapped to the same region of chromosome 13 as did HSCR2. Among the

Mennonites, at least 5 megacolon patients did not seem to carry the

specific EDNRB mutation trp276 to cys (W276C) present in most of the

affected members. This suggested the existence of as yet undiscovered

HSCR susceptibility genes. Among the affected Mennonites, HSCR was

associated with nonenteric phenotypes in several: bicolored irides

(6.3%), hypopigmentation (2.5%), sensorineural hearing loss (5.1%),

and white forelock (7.6%), reminiscent of the Shah-Waardenburg

syndrome [see also 22q] Puffenberger et al. suggested that these

nonenteric features represented pleiotropic effects of the W276C

mutation. This and other findings indicated that interaction of EDN3

with the endothelin-B receptor is essential to the development of

neural crest-derived cell lineages.4

16p

[The values given for 16p (IMGSAC 1999 1.51, pe et al. 1998

0.74) are scores below 3 but is in a region cited in more than one study.]

Defects associated with chromosome 16p include the differential

diagnosis of the guttate leukoderma of TSC (Tuberous Sclerosis), also

found on chromosome 9q and includes several clinical entities such as

idiopathic guttate hypomelanosis, 18 disseminated hypopigmented

keratoses, and dyschromic amyloidosis. 8, 9, 10, 11

18q

Defects associated with chromosome 18q include Erythropoietic

Porphyria. B-Cell CLL/Lymphoma (unknown inhibitor of apoptosis)

dominant white-spotting; ?B-cell lymphomas and polyclonal B-cell

lymphoproliferative disorders are related to Epstein-Barr virus (EBV)

activation.?83

19p

Defects of chromosome 19p include: Cytochrome P450: CYP4F3,

Leukotriene B4 omega-hydroxylase (LTB4H) bioactive compounds that play

roles in such processes as inflammation. Fucosyltransferase deficiency

(co-occurring condition - enzyme deficiencies) potentially relating to

purine and pyrimidine biosynthesis. Oligodendrogliomas (tumors

associated with ataxia, visual impairment and seizures) 5, 6 [see also 1p]

Regions not cited in the whole genome studies but with literature to

support the connection to ASD:

Chromosome 15 ?The strength of evidence supporting claims of a

specific association between genetic /chromosomal disorder and ASDs is

very variable. The strongest evidence of a causal association is found

for tuberous sclerosis, fragile X and inverted duplications of

chromosome 15. While fragile X Syndrome used to be thought to affect

as many as 25% of males with ASDs, Fombonne, in his review estimated a

far lower figure (0.75%).The symptoms shown by those with this

disorder may be more properly described as ?autism like? or fitting

only within the broader spectrum. An association between duplications

and triplications of part of the long arm of chromosome 15 of maternal

origin has been found with ASDs, often accompanied by severe mental

retardation. This is of some interest as genetic studies of ASD have

identified loci on the long arm of Chromosome 15. The role of mental

retardation in the association needs to be investigated. In addition,

sex chromosome abnormalities ( syndrome) have been linked to

ASDs. Some 5% of females with syndrome have either ASDs or

features that may fall within the broader ASD phenotype. In all

confirmed cases the normal X chromosome was maternal in origin. It is

possible that there is increased vulnerability to ASDs in females who

lack a normal paternally derived X-chromosome. Untreated

phenylketonuria is nowadays so rare that the evidence for an

association stems from very early studies that were undertaken before

the use of well-developed and validated diagnostic criteria, although

the evidence from the early reports is quite persuasive. The frequency

of single gene disorders or chromosomal abnormalities in population

based and clinic samples of individuals with ASDs is low and amounts

to at most 5-10% of the population.? 1 Additional defects associated

with chromosome 15 include: Cytochrome P450 ?aldehyde dehydrogenase?,

?retinaldehyde dehydrogenase 2?. Chromosome 15 syndrome of inv dup

(inversion-duplication): Macrogenitalia, seizures, autism. 80

15q Defects associated with chromosome 15q include: Cytochrome P450

?molybdenum cofactor deficiency? molybdenum cofactor is essential to

the function of 3 enzymes: sulfite oxidase, xanthine dehydrogenase and

aldehyde oxidase. Molybdenum cofactor (molybdopterin) deficiency is

characterized by developmental delay, seizures, lens dislocation and

often early infantile death; CYP19: ?estrogen synthetase?, induced by

follicle stimulating hormone; CYP450

?polycystic ovary syndrome?; CYP1A2 (deficiency: porphyria cutania

tarda); CYP11A, cholesterol side chain cleavage; CYP1A1: (XRE?s)

xenobotic response elements. Oculocutaneous albinism type 2 (OCA2),

?Xanthism type (OCA3) found on chromosome 9q?, [?The P protein, with

12 transmembrane domains localized to the melanosome, has no assigned

function as of yet but is responsible for OCA2 when dysfunctional?3]

pink protein, melanosomal membrane. 30, 35, 38, 40, 42, 43 Angelman

?light pigmentation? and Prader-Willi characterized by obesity, round

face, almond shaped eyes, obsessive compulsive eating and often with

scratching and itching.

4q Defects associated with chromosome 4q include Cytochrome P450

?Circadian Locomotor Output Cycles Kaput? CLOCK (Steeves et al 1999).

Piebaldism c-kit receptor: congenital patches of white skin and white

hair, principally located on the scalp, forehead, chest and abdomen

and on the limbs; several patients report lifelong severe constipation. 23

We have within our case histories an adult male age 40 who was

diagnosed with Down Syndrome, ASD and piebaldism. He was quite high

functioning and generally healthy. He did exhibit the characteristic

self-limited diet of the Down syndrome- ASD group. His diet mainly

consisted of pork sausages, mashed potato, fruit yogurt, wheatabix

cereal with milk, hot tea with milk and occasionally mashed

cauliflower, fish or chocolate. When hungry enough he could be coerced

into eating the mashed potato with pureed vegetable (usually peas,

carrots or broccoli). After these meals he would become withdrawn and

complain of headache this might be followed by a period of 1 or more

days when he would refuse food altogether. He was childlike and mildly

mischievous. He attended some special college courses, participated in

some social activities and was adapting to a supported living

arrangement. His medical history was conspicuously lacking any

reference to other medical conditions which often co-occur with Down

syndrome. He also had the wide gap between the first and second toes

(big toe and long toe) characteristic for Down Syndrome 111 and

prevalent in autism. Twenty nine of the 35 children we saw in India

had this wide gap between the toes.

10q Defects associated with chromosome 10q include: Cytochrome P450:

(RBP) retinal-binding protein; CYP2E (ethanol-inducible P450);

CYP45026A2, CYPP450RAI2 (retinoic acid inactivating) capable of

converting all-trans RA to polar metabolites. Retinoic acid (RA) plays

an important role in regulating gene expression during embryonic

development; CYP2C19, mephenytoin-4-Pime-hydroxylase; CYPC17,

?congenital adrenal hyperplasia? due to 17-alpha hydroxylase

deficiency; CYP2C9, CYP2C8, CYP2C18 (gene expression).

Oligodendrogliomas ?pterin-4-alpha-carbinolamine dehydratase? (PCBD)

leads to mild PKU ?hyperphenylalaninemia?, hyperphenylalaninemia with

premapterinuria (increased ratio of neopterin to biopterin). Hermansky

Pudlak syndrome: Hermansky-Pudlak Syndrome (HPS) is a genetic

metabolic disorder which causes albinism, visual impairment, a

platelet dysfunction with prolonged bleeding, and progressive symptoms

including pulmonary fibrosis, inflammatory bowel disease and kidney

disease. The severity of HPS ranges from very mild with few symptoms

to severe and disabling., membrane protein lysosome/melanosome

structure/function. Hermansky-Pudlak syndrome is characterized by

oculocutaneous albinism, a storage-pool deficiency, and lysosomal

accumulation of ceroid lipofuscin, which causes pulmonary fibrosis and

granulomatous colitis in some cases. 16

17q Defects associated with chromosome 17q include: café-au-lait

macules (co-ocurring condition ? asthma); 2, 46 Café-au-lait macules

are commonly reported by parents among the many types of pigment

anomalies described for ASD children. Autosomal dominant retinitis

pigmentosa (ADRP) (Inglehearn CF et al). Sanfilippo (sulfate sulfatase

deficiency and sulfamidase deficiency).

Chromosome 21, (not included in the genome screen cited in the table)

Down Syndrome (trisomy 21) is estimated by the University of

Goteberg, Sweden, to co-occur with ASD at 5 to 9% and by Kennedy

Kreiger at 5 to 7 % which also cites Down Syndrome co-occurring with

epilepsy at 10%. 92, 93 These figures, rarely cited in the literature,

indicate that as many as 2% of the autism population also have Down

syndrome.

22q Defects associated with chromosome 22q include: Cytochrome P450:

?peroxisome proliferator-activated receptor-alpha?(PPARA); CYP2D,

?xeno-biotic metabolism?, ?thyroid-lupus autoantigen?; CYP3A7,

?Polypeptide 7?, CYP3A5 gene constitutes the major genetic determinant

of polymorphic activity in humans. (ussen, A et al, 2000).

Adenylosuccinate lyase deficiency. Waardenburg-Shah Syndrome: Dystopia

canthorum (lateral displace-ment of the inner canthus of each eye)

Pigmentary abnormalities of hair, iris, and skin (often white forelock

and heterochromia iridis); Sensorineural deafness. The combination of

recessively inherited WS type II characteristics with Hirschsprung

disease has been called Waardenburg-Shah syndrome or WS type IV,

SRY-box containing gene 10 transcriptional activator, Mutations of

SOX10. Hearing impairment. 27, 41, 86 See also 2q (Hirschsprung is

also associated with chromosome 13 and 20)

Xp Defects of chromosome X include: Cytochrome P540 ?granulomatous

disease?, chronic CGD (lack of metabolic burst). Ocular albinism type

1, G-protein-coupled receptor signal transduction Mutations of OA1,

pinkeyed-dilution (p). 30, 32, 68 Incontinentia pigmenti (IP)

(usually lethal prenatally in males): 9 ?We show the complications

observed in a large series of children with Hypomelanosis of Ito (HI)

or incontinentia pigmenti achromians, studied in a neurology service

over 30 years. Of the 76 patients, 35 were male (46%) and 41 female

(54%) with ages ranging from newborn to 10 years at the time of the

first visit. Mental retardation was observed in 43 cases (57%) of whom

eight (10%) showed autistic behavior; 16 (21%) were borderline and

only 17 (22%) had a normal mental level (IQ > 85). Thirty-seven

patients (49%) had seizures, consisting of infantile spasms in six

cases (8%). Twelve cases showed macrocephaly and coarse facies, six

had microcephaly, and 14 showed hypotonia with pes valgus and genu

valgus. Three cases of cerebellar hypoplasia, another of intracranial

arteriovenous malformation and another of distal spinal muscular

atrophy were observed as well. Some other anomalies, such as

syndactyly, clinodactyly, abnormalities of the skeleton, asymmetry of

the facies, ears, body and/or extremities, gynecomastia and

asymmetrical breasts, short stature, oral alterations, congenital

cardiopathies and genital anomalies, were also occasionally found.

Three children died, but necropsy was performed only in one.

Anatomical and histological studies did not disclose specific

findings.? 17 ?It is suggested that skin changes indicating underlying

neurocutaneous disorders be meticulously looked for in all cases with

autism and autistic-like conditions.? 21

?Subcellular defects of hypomelanosis in tuberous sclerosis (TS) (28

subjects) were compared by light and electron microscopy with other

forms of congenital circumscribed hypomelanosis that occur in nevus

depigmentosus (ND) (8 subjects) and in piebaldism (PB) (4 subjects),

respectively.? 22 ?Embryologic migration of both melanoblasts from

neural crest and cortical neurons occurs in the second trimester,

suggesting a common mechanism for the developmental pathology of skin

and brain.? 24

Four adjacent loci, C4A, 21-OHA, C4B, and 21-OHB located in the class

III region of the major histocompatibility complex (MHC).

In this region, known as the hypervariable region or the mitochondrial

DNA region, the 21-OHB gene is generally inactive. The C4A gene

abnormalities are reported to be more common in the parents of

autistic offspring and particularly the mothers. The C4B gene null

allele is more frequent in the autism population and appears to

increase immune reactivity to viral pathogens, toxins, molds, pollens

and other hapten substances. The immune system evolution to include

plant pigments as pathogens has likely been exacerbated by the use of

plant substance in vaccines. Likewise, there is evidence that the

autistic population cannot make enzymes from plant foods in the normal

way. In the human body bacteria such as e-coli, candida and fungi

species produce these substances (carotenoids) for us. As humans we

have evolved to include in our gut flora species such as lacto

bacillus acidophilus (dependant on lactose, milk sugar) for some of

our vitamin manufacture (particularly some B vitamins). In earlier

human history it is not reasonable to expect that humans required

lactobacillus acidophilus because milk was not regularly consumed

other than in infancy through breastfeeding which primarily provides

bifidus.

It appears that people who have anomalies among these four adjacent

loci are the population referred to as having immune system disorders.

Offspring from families who have these anomalies have a greater chance

of being diagnosed with autism. It also appears that in autism, enzyme

deficiencies without any corresponding genetic anomaly is a prevalent

finding.

In the human body, Cytochrome P450 is involved in the manufacture of

enzymes and is strongly influenced by Fe2+ ascorbate (Iron + Vitamin

C). The breakdown products from plant foods particularly carotenoids,

are used for the formation of mevalonic acid in a normal metabolism,

which in turn is used in the regulation of cholesterol. Cholesterol

is the primary regulator of hormones. In autism Cytochrome P-450

enzymes are deregulated as the immune system removes the pigment

component and releases the other plant by-products. This interference

or regulation of pigments by the immune system results in changes in

the body of an autist which we see as enzyme deficiencies, allergic

reactions, skin pigmentation anomalies, dermatological irregularities,

digestive problems, nutrient imbalance, gut dysbiosis, metabolic

imbalance. Thus autism can be called a disorder of mitochondrial DNA,

an immuno-genetic condition, an inborn error of metabolism, or a

pigmentary disorder.

NADPH P-450 is required for the breakdown and conversion of pterins

(pigments). In autism, some of these pigments are being engulfed by

immune macrophages and the conversion process is disrupted. This

results in findings such as elevated levels of neopterin and biopterin

in the urine. This also results in the manufacture of alkoxyl and

epoxy alkyl radicals. One potential by-product or release product from

the immune macrophages when engulfing the pigment lutein is aldehyde

which inactivates Cytochrome P-450.

?Genes encoding several serum complement components and the gene(s) for

steroid 21-hydroxylase (21-OH) have been located in the class III

region of the major histocompatibility complex (MHC). All these genes

are highly polymorphic in man, and these polymorphisms have been used

to draw conclusions about the structure and function of these genes.

For example, electrophoretic polymorphisms of the fourth component of

complement (C4) have been shown to be controlled by two closely linked

genes, which also control expression of the red cell antigens.

(Rodgers and Chido) Steroid 21-OH deficiency (D) can occur in several

forms which differ in severity, and because of genetic linkage

disequilibrium with different HLA antigens the inheritance of these

forms is consistent with the existence of several alleles at a single

locus. When severe 21-OH D occurs in association with the HLA

haplotype A3;Bw47;DR7, there is a simultaneous null allele at one of

the C4 loci. This was hypothesized to result from a single deletion or

rearrangement affecting the 21-OH and C4 loci and perhaps the HLA-B

gene as well. These experiments showed that at least one structural

gene for the cytochrome P450 specific for 21-hydroxylation is located

in the MHC, probably very near the C4 genes, and a mutation in this

gene results in 21-OH D. Cosmid clones have been used to locate the

21-OH genes both in man and mouse. In both species, there are two

21-OH genes, each located immediately 3' of one of the two C4 genes,

and oriented in the same direction as the C4 genes. In man, the gene

located 3' of the C4B gene is deleted in 21-OH D on the Bw47

haplotype, but the gene 3' of the C4A gene is deleted in hormonally

normal individuals on the A1;B8;C4AQO;C4B1;DR3 haplotype. Thus the

21-OH B gene is normally active in man, but the 21-OH A gene is not.? 41

4p Defects of chromosome 4p include: Hermansky-Pudlak syndrome (HPS).

Wolf-Hirschhorn syndrome: Clinical features include mental

retardation, seizures, distinct facial appearance, and midline closure

defects. 4 Parkinsonism.

5p Melanin-concentrating hormone (MCH) gene (Viale A et al). Defects

of chromosome 5p include: Primary ciliary dyskinesia, left-right

asymmetry (Olbrich H et al). ?The spectrum of heterotopic

calcification or ossification is expanding because of the reports of

several kindreds with calcium pyrophosphate deposition disease,

apatite deposition disease, and others with less common syndromes

associated with extracellular matrix calcification, such as

fibrodysplasia ossificans progressiva and related syndromes.? 3

Although calcification problems have been identified to coexist with

autism i.e. tuberous sclerosis and PKU, the genetic findings would

indicate a probable predisposition to arthritic conditions, and yet

these have not been reported in the literature. Potentially the immune

system involvement in purine and pyrimidine disturbances includes

removal of formed crystals via the skin rather than deposition,

possibly to protect the kidneys. The findings of an association of

chromosomes 5p (calcification) and 7q (hemochromatosis) in autism

research (7q in multiple genome studies) would tend to support the

findings of a pilot study ?Preliminary study of altered skin

temperature at body sites associated with self-injurious behavior in

adults who have developmental disabilities,? J. Symons, A.

Sutton, and W. Bodfish, American Journal on Mental Retardation,

Vol. 106, No.4, 2001. The article includes ?skin temperature changes

can be indicative of neuropathy . . . For each participant the body

site targeted most frequently for self-injury may itself change skin

temperature, evidence from other animal studies suggests that

neuropathy can lead to self-injury. For Instance, they note,

self-mutilation is a common finding in animals with dysesthesia, or

abnormal sensations caused by lesions of the sensory nerve pathways.

In addition, cases of intellectually normal individuals who targeted

self-injury toward areas with neuropathy and altered skin temperature

have been documented.

?The researchers suggest that self-injury may provide temporary relief

of neuropathy-caused pain or discomfort (as when a child scratches at

a scab or chicken pox lesion), but may lead in the long term to more

irritation that in turn generates more self-injury. We suggest that,

in autism, the pain and discomfort is caused not by sensory neuropathy

but by the immune system removal of calcium and other crystals as well

as other breakdown products of lutein (formic acid, epoxy residues)

through the skin.

?The researchers note that all four of their subjects responded well to

naltrexone, a substance that blocks opioid receptors and alters

perception of pain. They suggest that further studies examine whether

naltrexone treatment, although it has no known effect on neuropathy

itself, is more effective in individuals with skin temperature

differences at self-injury sites.

?In addition, Symonds and colleagues say, ?one clinical implication of

a pain related model of self-injurious behavior (SIB) is that

treatments for peripheral neuropathies may provide an effective

treatment option for some subset of SIB cases.? They note that several

studies show decreased self-injury during treatment with

transcutaneous electrical nerve stimulation (TENS), a treatment that

blocks peripheral pain signals.?? 45

Symptom versus behavior

In real terms, the combination of these processes - genetic, immune

and environmental: genetic predisposition, immune reaction and

environmental stimuli - result in the signs and symptoms which in

autism are called behaviors. Current research is beginning to provide

insights into what the autist is experiencing from his or her own

biochemistry, physiology and environment. We are beginning to

understand that the world of the autist is a little different and

their perception of the world is also quite different. Since I have

this lutein reaction in my own body, and have spoken to many autists

who have related their experiences to me, I feel that it is worthwhile

to contribute some information on how these scientific terms relate to

real life experiences.

Vision

Unlike some autists who are blind and others who are born blind and

then have visual maturation with the onset of autistic ?symptomology?

I was born blind in one eye. I did not obtain vision in my left eye

until my second pregnancy and the ophthalmologist suggested that

increased steroid hormones produced during pregnancy probably resulted

in the ?visual maturation? at the age of 21. To this I ask why are

steroid hormones not considered for this type of childhood visual

disturbance? The eye doctors had no trouble putting me through wearing

a patch over my sighted eye which resulted in extreme anxiety and then

stating there was nothing else he could do.

The vision I acquired in my left eye was partial, the depth perception

was quite different from my right eye. This also caused anxiety and so

I had to wear glasses. Since my right eye had exceptionally good

vision I did not see as well with glasses even though the right eye

lens was clear glass. So, I got a contact lens for my left eye. This

was somewhat helpful except when I took the contact lens out I got a

headache. Eventually I got an eye infection and steroid drops resulted

in better depth perception and I could go without glasses. With both

eyes open the visual images were from my right eye only. It was only

when I closed my right eye that I could see blurry images with my left

eye.

When I began the lutein free diet at the age of 35 my vision changed

within a year. I could see from my left eye clearly for the first time

and this was a strange experience. First I felt like I was very tall

looking way down to the ground. The next moment I might feel very

short as if I had shrunk to a midgets height. It took several days for

these visual distortions to stop. I met Dr. Gwen , trained as an

Irlen Lens diagnostician and also a psychologist in Atlanta. Dr.

was testing , my adopted autistic (declassified) 15 year old

daughter for Irlen lenses. I also had a screening and so did my

husband. My Irlen lenses are a combination of pink and blue for the

left eye and plain blue for the right eye. I haven?t gone outside

without the Irlen lenses since I got them 4 years ago.

As I read about autism and studied the individuals with autism and

began to write about autism it was frustrating to see how impersonal

words would seem when trying to describe autism. To say my vision is

not bilaterally synchronized doesn?t tell you how visual stimuli

coming in at different speeds from different sides affects me in real

life. It affects me as I react differently to someone or something

approaching me from the left side or the right side. It means I

consider this subconsciously when I choose my seating in a restaurant

or at a meeting. It means that when I am riding in a car the way the

traffic moves on one side with the passing scenery still on the other

side results in spontaneous panic reactions, hand-flapping and rigid

finger movements. I suspect that my visual perception is similar to

some of the autistic population and for others there are more severe

complications and for some less.

I take cod-liver oil to support my vision even though I do not maintain

a dairy free diet. I find it difficult to take in enough butter, fresh

cheeses, liver and fish to meet the RDI for vitamin A. It would be

nearly impossible for those on a dairy free diet.

Not directly related to vision but still related to the eye area are

the hair follicles of the eye lashes. Some of these, about 5 or 6, on

my right upper eye lid have follicles that fill with fluid cyclically.

The fluid can become hardened and the eyelash becomes painful. It

feels like a needle sticking into the area. So, I remove these

eye-lashes and experience immediate relief from the pain. So, the

psychologists can call it trichotillomania (known to co-occur with

autism) if they want to but I call it a rational reaction to pain.

So, now I am quite satisfied that my vision is as good as it can be

with the Irlen lenses. Before starting the diet, my vision was

strongly affected by a lutein (food) reaction. I lost depth perception

altogether and would have to hold my hand against the wall to move

towards a chair or bed where I remained until the worst of the

symptoms passed (distorted vision, loss of depth perception, auditory

processing difficulties, vice-grip type headache, profuse sweating,

nausea, burping followed by muscle contractions, vomiting, diarrhea

and urinary incontinence). Since I didn?t know what lutein was or

which foods contained lutein I didn?t know when this was going to happen.

In early childhood (infancy to age 5) I had a very limited

self-selective diet: tomato soup, potato chips and baby rice cereal

and I would drink coke, sprite and coconut milk. Naturally lutein

free, very limited dairy and not much wheat. Parental intervention or

rather forced feeding led to severe reactions but also resulted in my

finding more foods that I could tolerate: White bread as toast with

butter, chocolate chips (dark chocolate), whipped cream, cream cheese,

most meats and lettuce were safe foods. Foods that made me ill were

discovered one by one: green peas, egg yolk, spinach and strawberries.

These foods didn?t appear to have anything in common. They were not in

the same food group or the same food family. I studied the food

groups, the nutrition manuals, the chemistry books and still there was

nothing until I read ?The development and application of a carotenoid

database for fruits, vegetables, and multicomponent foods? by Jaspreet

et al, published in the Journal of The American Dietetic Association,

March 1993 (Vol.93). All these foods contained lutein and more foods

not listed in the fruits and vegetables article also contained lutein

(i.e. chicken fat). I was now armed with information and I produced a

lutein free, nutrient balanced diet right down to the individual amino

acid levels for daily intake. I noted what foods had to be in the diet

and what nutrients would have to be supplemented if the menu was not

adequate. I put my diet into action and have been well with a few

mishaps ever since.

As I studied how an immune response of this type might work in the

human body I was convinced that this same approach might help my

autistic foster daughter. As soon as the legal process of adoption was

complete I placed her, with her pediatricians permission, on the

lutein free diet. Sara blossomed at age 11, began speaking in

sentences, stopped toe-walking and lost 17 of the 18 characteristics

of autism which she had expressed until we implemented the diet

change. Thus the ?Sara?s diet? story began. One of the teenage

autistic females who implemented the lutein free diet later described

how her eyes felt when she was having a food reaction, she said ?It

felt like my eyes were melting?.

Hearing

According to standard testing measures my hearing is good. I can play

a musical instrument and not get thrown out of a church choir. I have

tinitus (vibratory), ?ringing in the ears? but to me the sound is best

described as the sound that crickets make, lots of crickets. I like

the sound. At times I have managed to restrict my diet enough that the

sound goes away completely but then I feel vulnerable as normal

everyday sounds become loud and painful. I can imagine that for some

people who develop tinitus they must experience the continual sound as

painful and annoying, maybe even unbearable at times.

A long time ago people who were diagnosed with Meniere?s disease ( a

type of deafness) were prescribed a carotenoids free diet. This was

before World Health Organization determined that carotenoids were good

for everyone (they didn?t do the research and they were wrong, the

research has since been done but no change in WHO policy has

occurred). Would the World Health Organization admit such an error and

make a retraction jeopardizing modern practices of putting more

carotenoids into our diet, adding beta-carotene to rice, increasing

the lutein production in potato and cucumber and supporting the

development of edible vaccines in carotenoids bases. I don?t think

they will. Nor does anyone expect them or science in general to

acknowledge the vaccine contribution to modern day disease. But the

promotions of the World Health Organization have impacted my life

tremendously. I tried to take into account their recommendations and I

suffered. Every time I ate a lutein containing food I became ill. One

of the symptoms was loss of auditory perception. People?s mouths would

be moving but I could not hear any sounds. The tinitus would intensify

and block out all incoming sound. Fortunately this was temporary and

when the headache went away my hearing returned.

I suspect that I like the tinitus because it helps me to tune out the

world. I can study and concentrate on my work better with the

continual hum. Sometimes a sound from the outside world penetrates and

the vibration from the sound is disturbing. By disturbing I mean that

my body panics and I cannot tolerate the noise, I become unwell,

nauseated and fearful. The sound of a paint compressor is one type of

sound which can produce this panic reaction. I will leave the area of

the sound and return when the sound is gone.

Smell

Some smells result in immune reactions, particularly anything with

pine oil (Christmas trees and cleaning products), some commercial

carpet shampoo, brass cleaner, finger nail polish, some perfumes,

deodorants, hair sprays, oil based paints. Often these are epoxy odors

or substances that contain pinene. The reaction to fumes is quite

different, usually intoxicating, euphoric even. Lengthy exposure can

be more intense resulting in crying for no apparent reason or

hysterical laughter. I have discovered that I am especially allergic

to the smell from pitocin. When the women on the labor ward were put

on pitocin IV?s I could not enter the ward and had to work in a

different area of the hospital, the reaction was respiratory,

asthma-like (coughing, wheezing, shortness of breath) and I do not

have asthma. The smell of fresh oranges being peeled results in an

intoxicating ?high?.

Taste

I am not aware of any issues with taste. I like the taste of most

foods but I do have sensory issues. Metal, such as forks and spoons

cannot touch my teeth. Only certain teeth actually touch the food.

Getting me to chew took some effort on my parents part. But I have

many autistic friends and one of them says ?Getting an autistic person

to eat something they don?t like is like trying to get a normal person

to eat a hairball?.

Touch

I guess I spent a lot of time examining my own skin, often with a

microscope. I was curious as to why I had blue ink like deposits in

the pores of my developing breasts, why I had hard white crystal-like

substances erupting from the pores on my upper arms, and what made

blue ear wax. Some people spent their teen years socializing and I

spent mine in the medical library. The textures which bother me the

most are cardboard, the cotton wool filler that comes inside medicine

bottles and some fabrics, particularly wool. I won?t wear anything

that is uncomfortable (too binding). Physical touch is good but I am

easily startled by unexpected touch.

My skin is good now with only occasional itching when I get too much

calcium in the diet or too much oxalates. I have experienced a lot of

phenomena with substances that are excreted through my skin, small

round white lumps which pop out and look like small pearls. These

don?t pop out easily and yet until they are out they are painful, they

feel like daggers under the skin. Usually the lumps are smaller like a

large grain of salt. Sometimes they are just the hair follicle wax

which has hardened and crystallized. A food reaction would result in

itching and burning which felt like being bitten all over by

mosquitoes with no visible rash. Sara?s reactions in the skin were

quite different. Often she would have 3 to 7 blisters which looked

like infected mosquito bites on her skin ? small clear to yellow

blisters which she scratched as soon as she could get to them. Since

being on the diet these no longer appear. I believe these are

substances released from the macrophages which are derived from plant

foods (triterpenoids, epoxides) which are agglutinated and removed (in

the autist) via the skin. I have seen many pictures of autists with

these ?prurigo? type eruptions.

My reaction to some types of pain is abnormal. I have experienced a

rush from head to toe resulting in euphoric type feelings from grease

burns and a broken foot. I had no pain when I had an appendicitis or

after the surgery. Other types of pain, particularly ovulation and

toothache are overwhelming at times.

Balance

My balance is generally good but I did practice walking with a book on

my head (grandmother?s suggestion). I was not good at sports, possibly

because of the visual problems but I was very good at deck tennis

(catching a rubber ring that came flying over a net). As far as sports

went I wasn?t the last one chosen, I wasn?t even an option. I was

designated as the instructors helper. I have experienced vertigo,

severe and disabling when it happens. It can be triggered by kaweol,

an oil in arabica coffee or from too much xanthine (coffee, tea,

chocolate or violaxanthine in purple vegetables). Violaxanthine can be

converted to zeaxanthine in the body, which may be needed to protect

the eyes, and works similar to lutein in the eyes. The lutein

containing foods are also generally zeaxanthine foods, so getting the

violaxanthine foods into the diet is important, if tolerated, to

provide another source of protection for the eyes. Lycopene is another

food that can be converted to photoprotective pigments in the body.

Once lutein has been out of the diet for 4 months, lycopene foods

(e.g. tomatoes) can usually be safely returned to the diet. Once the

autist is accommodated with the lutein free diet and he or she no

longer has the strong food related anxiety then it becomes easier to

get the non-lutein fruits and vegetables into their diet.

Well-being

As the term autism was first used to describe ?withdrawal from the

fabric of social life? it is easy to understand that people who are

not well withdraw from the fabric of social life. People who are not

well do not experience a sense of well-being and this is necessary to

move towards developing emotional maturity. People with autism, a

movement disorder suffer with symptoms which can be seen and heard as

well as hidden from view; dyskinesia: burping, jerking in the night,

hiccups, muscle contractions. These symptoms can and do interfere with

sleep and a sense of well being impeding our ability to develop

skills. The skills of emotional maturity are necessary for the

development of social skills. So, to move forward with an autistic

individual one must look at the world through their eyes, hear the

world differently, look at foods differently. The environment has to

meet their needs. The tools must be modified and adapted to meet their

needs. Habilitating a generation of autists is not going to be easy.

It will take people with foresight, insight and fortitude. Most of the

autistic population will have strength and determination (it takes a

lot of determination to limit one?s own diet to the minimal foods many

of these individuals accept). We have to help them learn to use their

strengths rather than holding them back through their weaknesses. Just

because some won?t be able to speak doesn?t justify not teaching them

to read. Just because many write in large scrawling letters doesn?t

mean they can?t learn to write on a keyboard.

The consistency of the findings in autism: relationship to pigment

metabolism, immune and genetic findings, consistent markers i.e.

transferase deficiencies, elevated GFAP, urinary metabolites i.e.

elevated neopterin, biopterin, IAG and serum serotonin levels strongly

suggest that symptoms which occur with consistent biochemical, immune

and IoGc markers would indicate a common causal factor.

Desorgher BSc, BSC, MA-TFM, NR, NM

http\\:www.saras-autism-diet.freeservers.com/

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