*** RESEARCH: MSA MOUSE MODEL DEVELOPED ***

[Guest guest]

More exciting news! This was sent to me by one of my friends at the

National Ataxia Foundation.

Hugs,

Pam

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

To all people interested in MSA/Shy-Drager Syndrome:

Below is an interesting news release of 10 June 2002.

Carl Lauter

Chesapeake Chapter - NAF

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

Information from: Foundation for Biomedical Research,

E-Clips #165, June 10, 2002

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

Mouse model developed for widespread neural disease

EurekAlert, June 9 - Researchers have developed a mouse model

to show pathological symptoms similar to those of human patients

suffering from the neural disease Multiple System Atrophy (MSA), also

known as Shy-Drager-Syndrome.

The model could help researchers to develop and test efficient

new drugs against this widespread disease. Read more. [see below]

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

Public release date: 10-Jun-2002

Contact: Dr. Christian Haass

chaass@...

49-89-5996-472

European Molecular Biology Laboratory

Mimicking a human disease in mice

A new model for the Parkinson related illness multiple system

atrophy

In this month's issue of EMBO Reports Kahle et al. describe how

they genetically engineered a mouse to show pathological symptoms

similar to those of human patients suffering from the neural disease

Multiple System Atrophy MSA), also known as Shy-Drager-Syndrome. The

model could help researchers to develop and test new efficient drugs

against this wide spread disease.

More than 100,000 Europeans and 100,000 US-Americans suffer

from MSA. Affected individuals either show symptoms similar to those

of patients suffering from Parkinson's Disease or have a strong

deterioration in their sense of balance. For this reason the disease

is often diagnosed incorrectly. Doctors know very little about the

pathology of the disease. However, one characteristic is that some

brain cells show abnormal changes. Affected mature oligodendrocytes,

the ells that form the isolating outer layer surrounding nerve

fibers, produce a small protein called alpha-synuclein. They

deposit this protein in the form of pathological structures

called glial cytoplasmic inclusions.

Healthy mature oligodendrocytes do not produce this protein at

all.

Kahle and colleagues " implanted " the human gene for the

alpha-synuclein protein into the mouse genome. As a result, the

researchers found insoluble inclusion bodies of alpha-synuclein in

the mouse's oligodendrocytes. " In patients, the affected cells die as

the individual ages. This is something we could not yet observe in

our mice, " says Philipp Kahle, a researcher at the Ludwig Maximilian

University, Munich, Germany. " But we are confident that in a next

step we can produce mice that will also show this symptom. This will

help us to understand more about the disease and can help

researchers to develop and test drugs against multiple system

atrophy. "

###

Prof. Dr. Christian Haass

Abteilung für Biochemie

Adolf-Butenandt-Institut

Ludwig-Maximilians-Universität

Schillerstrasse 44

80336 München

Tel.: (089) 5996-472 (sekr.)

Fax: (089) 5996-415

E-mail: chaass@...

For a pdf of the paper please contact:

ellen.peerenboom@...

===================================================

[Guest guest]

Pam,

That IS good news. Sounds like they still have a bit

of work to do to make it usable, but I'm greatly

encouraged that someone is actually working to develop

such a model.

Carol & Rob

Lexington, MA

--- pammygbower pbower@...> wrote:

>

> More exciting news! This was sent to me by one of

> my friends at the

> National Ataxia Foundation.

>

> Hugs,

> Pam

>

> -------------

>

> To all people interested in MSA/Shy-Drager Syndrome:

>

> Below is an interesting news release of 10 June

> 2002.

>

> Carl Lauter

> Chesapeake Chapter - NAF

>

> ------------------------------------------

> Information from: Foundation for Biomedical

> Research,

> E-Clips #165, June 10, 2002

>

> ----------------------------------------------

> Mouse model developed for widespread neural disease

> EurekAlert, June 9 - Researchers have

> developed a mouse model

> to show pathological symptoms similar to those of

> human patients

> suffering from the neural disease Multiple System

> Atrophy (MSA), also

> known as Shy-Drager-Syndrome.

> The model could help researchers to develop

> and test efficient

> new drugs against this widespread disease. Read

> more. [see below]

>

> ---------------------------------

> Public release date: 10-Jun-2002

>

> Contact: Dr. Christian Haass

> chaass@...

> 49-89-5996-472

> European Molecular Biology Laboratory

>

> Mimicking a human disease in mice

>

> A new model for the Parkinson related illness

> multiple system

> atrophy

>

> In this month's issue of EMBO Reports Kahle

> et al. describe how

> they genetically engineered a mouse to show

> pathological symptoms

> similar to those of human patients suffering from

> the neural disease

> Multiple System Atrophy MSA), also known as

> Shy-Drager-Syndrome. The

> model could help researchers to develop and test new

> efficient drugs

> against this wide spread disease.

>

> More than 100,000 Europeans and 100,000

> US-Americans suffer

> from MSA. Affected individuals either show symptoms

> similar to those

> of patients suffering from Parkinson's Disease or

> have a strong

> deterioration in their sense of balance. For this

> reason the disease

> is often diagnosed incorrectly. Doctors know very

> little about the

> pathology of the disease. However, one

> characteristic is that some

> brain cells show abnormal changes. Affected mature

> oligodendrocytes,

> the ells that form the isolating outer layer

> surrounding nerve

> fibers, produce a small protein called

> alpha-synuclein. They

> deposit this protein in the form of pathological

> structures

> called glial cytoplasmic inclusions.

>

> Healthy mature oligodendrocytes do not

> produce this protein at

> all.

>

> Kahle and colleagues " implanted " the human

> gene for the

> alpha-synuclein protein into the mouse genome. As a

> result, the

> researchers found insoluble inclusion bodies of

> alpha-synuclein in

> the mouse's oligodendrocytes. " In patients, the

> affected cells die as

> the individual ages. This is something we could not

> yet observe in

> our mice, " says Philipp Kahle, a researcher at the

> Ludwig Maximilian

> University, Munich, Germany. " But we are confident

> that in a next

> step we can produce mice that will also show this

> symptom. This will

> help us to understand more about the disease and can

> help

> researchers to develop and test drugs against

> multiple system

> atrophy. "

>

> ###

>

> Prof. Dr. Christian Haass

> Abteilung für Biochemie

> Adolf-Butenandt-Institut

> Ludwig-Maximilians-Universität

> Schillerstrasse 44

> 80336 München

> Tel.: (089) 5996-472 (sekr.)

> Fax: (089) 5996-415

> E-mail: chaass@...

>

> For a pdf of the paper please contact:

> ellen.peerenboom@...

>

> ===================================================

>

>

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