Document Detail


Acute multiple sclerosis (Marburg type) is associated with developmentally immature myelin basic protein.
MedLine Citation:
PMID:  8687186     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We have studied a case of acute, fulminating multiple sclerosis (MS) (Marburg type) at the pathological and biochemical levels. Postmortem examination of the brain revealed extensive areas of gross rarefaction in the hemispheric white matter. Histologically, well-demarcated areas of demyelination with a large influx of macrophages and a subtle perivascular infiltration of lymphocytes were seen with relative preservation of the axis cylinders. Myelin basic protein (MBP) was isolated and purified [correction of purifed] from noninvolved white matter. It was slightly larger in molecular weight than MBP from normal brain or from chronic MS brain. The increase in mass was accounted for, in part, by the deimination of 18 of 19 arginyl residues to citrulline, making the patient's MBP much less cationic than MBP from normal white matter. When expressed as the ratio of least cationic form of MBP to the most cationic (C-8/C-1), the normal ratio was 0.82, chronic MS 2.5, and the patient in this study 6.7. Because the ratio of 6.7 was similar to 7.5 found for a 15-month-old infant, MBP was considered to be of the immature form. The data are consistent with a genetic factor influencing the charge microheterogeneity of MBP. The resulting less cationic MBP cannot carry out its normal function of compacting multilayers.
Authors:
D D Wood; J M Bilbao; P O'Connors; M A Moscarello
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Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Annals of neurology     Volume:  40     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  1996 Jul 
Date Detail:
Created Date:  1996-08-21     Completed Date:  1996-08-21     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  18-24     Citation Subset:  IM    
Affiliation:
Division of Biochemistry, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada.
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MeSH Terms
Descriptor/Qualifier:
Adult
Antibodies, Monoclonal
Arginine / analysis
Blotting, Western
Brain / physiopathology
Brain Chemistry
Chronic Disease
Citrulline / analysis
Demyelinating Diseases / physiopathology
Fatal Outcome
Female
Humans
Lymphocytes / ultrastructure
Macrophages / ultrastructure
Magnetic Resonance Imaging
Multiple Sclerosis / diagnosis*,  physiopathology
Myelin Basic Proteins* / ultrastructure
Chemical
Reg. No./Substance:
0/Antibodies, Monoclonal; 0/Myelin Basic Proteins; 372-75-8/Citrulline; 74-79-3/Arginine

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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