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A skin biopsy can test for all the complexes except Complex 1 deficiency.

You would have to have a muscle biopsy to check your complex 1 activity.

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In a message dated 6/27/2005 10:57:19 AM Eastern Standard Time,

wheatchild@... writes:

Actually, I believe all the inborn errors in fat

metabolism (mitochondrial beta oxidation) can be diagnosed with a skin

biopsy, assuming the right person is doing the assays. J.

PhD in Texas is the fibroblast guru. Fibroblasts can be shipped, so there is

no need to go to Texas.

Dr is at The Children's Hospital of Philadelphia now. I think he

moved there last fall, so his lab is there now, as well. Dr Enns at Stanford in

CA is also doing testing with skin fibroblasts by looking at the complexes in

a new way...something about looking at or using antibodies which has never

been done before. I didn't really understand it, but it sounds really

interesting.

Malisa

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In a message dated 6/27/2005 10:57:19 AM Eastern Standard Time,

wheatchild@... writes:

Actually, I believe all the inborn errors in fat

metabolism (mitochondrial beta oxidation) can be diagnosed with a skin

biopsy, assuming the right person is doing the assays. J.

PhD in Texas is the fibroblast guru. Fibroblasts can be shipped, so there is

no need to go to Texas.

Dr is at The Children's Hospital of Philadelphia now. I think he

moved there last fall, so his lab is there now, as well. Dr Enns at Stanford in

CA is also doing testing with skin fibroblasts by looking at the complexes in

a new way...something about looking at or using antibodies which has never

been done before. I didn't really understand it, but it sounds really

interesting.

Malisa

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As I understand it, a skin biopsy is useful for some mito disorders, but not

others. I haven't read the medical literature on this recently, but five

years ago, I believe the consensus on mitochondrial respiratory chain

disorders was that while complex II deficiencies are reliably expressed in

cultured skin fibroblasts, deficiencies in complex I, III and IV may not

show up in fibroblasts. I don't know whether this position has changed in

the last few years, but I suspect not. For mitochondrial respiratory chain

disorders (OXPHOS), the preferred methods of diagnosis are either by

identifying the genetic defect via genetic testing or by identifying

deficiencies in one of the complexes via muscle tissue. However, other kinds

of mito disorders can usually be diagnosed in cultured skin fibroblasts. CPT

II deficiency is one. Actually, I believe all the inborn errors in fat

metabolism (mitochondrial beta oxidation) can be diagnosed with a skin

biopsy, assuming the right person is doing the assays. J.

PhD in Texas is the fibroblast guru. Fibroblasts can be shipped, so there is

no need to go to Texas.

As I recall, at least one former member of this group, Beth, had a complex

II deficiency diagnosed by skin biopsy. There may be others? The reason

Complex II can be diagnosed this way is that all the enzymes in complex II

are encoded in nuclear DNA, not mitochondrial DNA. The enzymes in I, III and

IV are a mixed bag---some are nDNA encoded, others are mtDNA encoded. In

those cases, its more difficult to confirm diagnosis by skin biopsy. That is

my understanding, at least. If anyone knows differently, please correct me.

I've not followed this issue recently.

Barbara

_____

From: [mailto: ] On Behalf

Of klaga5

Sent: Monday, June 27, 2005 9:02 AM

To:

Subject: skin or muscle biopsy

Have many of you been diagnosed by having a skin biopsy? Is it a good

alternative, or just a small chance of diagnosis? I'd love to avoid

another muscle biopsy, but want a diagnosis more.

Medical advice, information, opinions, data and statements contained herein

are not necessarily those of the list moderators. The author of this e mail

is entirely responsible for its content. List members are reminded of their

responsibility to evaluate the content of the postings and consult with

their physicians regarding changes in their own treatment.

Personal attacks are not permitted on the list and anyone who sends one is

automatically moderated or removed depending on the severity of the attack.

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Thanks, . In that case, a skin biopsy would be a good option.

B

_____

From: [mailto: ] On Behalf

Of LILQT4U1984@...

Sent: Monday, June 27, 2005 9:28 AM

To:

Subject: Re: skin or muscle biopsy

A skin biopsy can test for all the complexes except Complex 1 deficiency.

You would have to have a muscle biopsy to check your complex 1 activity.

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Thanks, . In that case, a skin biopsy would be a good option.

B

_____

From: [mailto: ] On Behalf

Of LILQT4U1984@...

Sent: Monday, June 27, 2005 9:28 AM

To:

Subject: Re: skin or muscle biopsy

A skin biopsy can test for all the complexes except Complex 1 deficiency.

You would have to have a muscle biopsy to check your complex 1 activity.

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I'm not sure what to do. I've asked my MDA doc, and my primary what

they think. I haven't heard yet. I also asked the MDA doc to test

the muscle they already have for mito. I understand because it's

frozen the testing is very limited. If he'd diagnose me with mito

in general, I'd probably leave it at that for now.

I'm still not sure if the skin biopsy would be worth it.

Kim

> Thanks, . In that case, a skin biopsy would be a good

option.

>

>

>

> B

>

>

>

> _____

>

> From: [mailto: ]

On Behalf

> Of LILQT4U1984@a...

> Sent: Monday, June 27, 2005 9:28 AM

> To:

> Subject: Re: skin or muscle biopsy

>

>

>

> A skin biopsy can test for all the complexes except Complex 1

deficiency.

> You would have to have a muscle biopsy to check your complex 1

activity.

>

>

>

>

>

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Hi Kim,

The reason I suggested that you think about having a skin biopsy instead of

going for another muscle biopsy was to give you some down time. Some time

to take a break from all of this medical stuff. A skin biopsy is simple and

easy and can be shipped to were it needs to go, which will free you up from

making any long distance trips right now.

It was just a thought.

Hugs,

Ann-Marie

At 12:35 PM 6/27/2005, you wrote:

>I'm not sure what to do. I've asked my MDA doc, and my primary what

>they think. I haven't heard yet. I also asked the MDA doc to test

>the muscle they already have for mito. I understand because it's

>frozen the testing is very limited. If he'd diagnose me with mito

>in general, I'd probably leave it at that for now.

>I'm still not sure if the skin biopsy would be worth it.

>Kim

>

>

> > Thanks, . In that case, a skin biopsy would be a good

>option.

> >

> >

> >

> > B

> >

> >

> >

> > _____

> >

> > From: [mailto: ]

>On Behalf

> > Of LILQT4U1984@a...

> > Sent: Monday, June 27, 2005 9:28 AM

> > To:

> > Subject: Re: skin or muscle biopsy

> >

> >

> >

> > A skin biopsy can test for all the complexes except Complex 1

>deficiency.

> > You would have to have a muscle biopsy to check your complex 1

>activity.

> >

> >

> >

> >

> >

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Hi Kim,

The reason I suggested that you think about having a skin biopsy instead of

going for another muscle biopsy was to give you some down time. Some time

to take a break from all of this medical stuff. A skin biopsy is simple and

easy and can be shipped to were it needs to go, which will free you up from

making any long distance trips right now.

It was just a thought.

Hugs,

Ann-Marie

At 12:35 PM 6/27/2005, you wrote:

>I'm not sure what to do. I've asked my MDA doc, and my primary what

>they think. I haven't heard yet. I also asked the MDA doc to test

>the muscle they already have for mito. I understand because it's

>frozen the testing is very limited. If he'd diagnose me with mito

>in general, I'd probably leave it at that for now.

>I'm still not sure if the skin biopsy would be worth it.

>Kim

>

>

> > Thanks, . In that case, a skin biopsy would be a good

>option.

> >

> >

> >

> > B

> >

> >

> >

> > _____

> >

> > From: [mailto: ]

>On Behalf

> > Of LILQT4U1984@a...

> > Sent: Monday, June 27, 2005 9:28 AM

> > To:

> > Subject: Re: skin or muscle biopsy

> >

> >

> >

> > A skin biopsy can test for all the complexes except Complex 1

>deficiency.

> > You would have to have a muscle biopsy to check your complex 1

>activity.

> >

> >

> >

> >

> >

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Glad to know Dr. has moved. Also the news about the work Dr. Enns is

doing with fibroblasts sounds very interesting. Keep us posted if you learn

more.

Barbara

_____

From: [mailto: ] On Behalf

Of Malilibear@...

Sent: Monday, June 27, 2005 12:23 PM

To:

Subject: Re: skin or muscle biopsy

In a message dated 6/27/2005 10:57:19 AM Eastern Standard Time,

wheatchild@... writes:

Actually, I believe all the inborn errors in fat

metabolism (mitochondrial beta oxidation) can be diagnosed with a skin

biopsy, assuming the right person is doing the assays. J.

PhD in Texas is the fibroblast guru. Fibroblasts can be shipped, so there

is

no need to go to Texas.

Dr is at The Children's Hospital of Philadelphia now. I think he

moved there last fall, so his lab is there now, as well. Dr Enns at Stanford

in

CA is also doing testing with skin fibroblasts by looking at the complexes

in

a new way...something about looking at or using antibodies which has never

been done before. I didn't really understand it, but it sounds really

interesting.

Malisa

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's skin biopsy evaluated at Mayo Clinic was positive for a Complex I

Defect. Mayo is using a new technique that they believe is better at evaluating

Complex I, III, and IV activity. I can ask Dr. Whiteman about it tomorrow if

you'd like. We are currently at Mayo Clinic for appointments tomorrow.

LILQT4U1984@... wrote:A skin biopsy can test for all the complexes except

Complex 1 deficiency.

You would have to have a muscle biopsy to check your complex 1 activity.

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In a message dated 6/27/2005 7:49:04 PM Eastern Standard Time,

servedogmom@... writes:

's skin biopsy evaluated at Mayo Clinic was positive for a Complex I

Defect. Mayo is using a new technique that they believe is better at

evaluating Complex I, III, and IV activity. I can ask Dr. Whiteman about it

tomorrow if you'd like. We are currently at Mayo Clinic for appointments

tomorrow.

Hi Joanne,

I'd be interested in hearing if they test for Complex 1 in other skin

biopsies. My skin biopsy was sent to Toronto and we're waiting to hear the

results.

What do you think of Dr Whiteman?

I don't think I've introduced myself to you...I'm 27 yrs old and have had

symptoms, which we now know are due to mito, since the age of 14. I have

significant GI dysmotility and have been on TPN since age 16, and started

developing autonomic symptoms, muscle symptoms, and significant fatigue issues.

a and I talk about how difficult it is to have mito at this age b/c we

seem

to be a minority. There are adults older than us and children younger than

us but not many our age which makes it hard for us to know what our future

holds. What ages did your girls get sick at?

Malisa

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