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Folia Neuropathologica
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4/2007
vol. 45
 
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abstract:

Myelin composition of spinal cord in a model of amyotrophic lateral sclerosis (ALS) in SOD1G93A transgenic rats

Irena Niebroj-Dobosz
,
Janina Rafałowska
,
Anna Fidziańska
,
Roman Gadamski
,
Paweł Grieb

Folia Neuropathol 2007; 45 (4): 236-241
Online publish date: 2007/12/21
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We present the results of biochemical and electron microscopic (EM) examinations of the spinal cord myelin from SOD1G93A transgenic Sprague Dawley rats in the early and late symptom-free period of the disease (60 and 93 days of life) and after four-leg paralysis has occurred (120 days of life). Biochemical and ultrastructural changes of myelin started already in the symptom-free period and become most pronounced in the paralyzed animals. Biochemical examinations indicated a decrease of lipids, phospholipids, cholesterol and cerebrosides. The pattern of particular phospholipids was in the normal range. A progressive decrease of the percentages of proteolipid, DM-20
and Wolfgram proteins was evident. Myelin basic proteins I and II were less affected. In EM, massive myelin disorganization was observed.
keywords:

amyotrophic lateral sclerosis model, myelin composition
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