Document Detail

The sialic acid and galactose concentrations in erythrocyte membranes in patients with myotonic dystrophy, limb-girdle and facioscapulohumeral dystrophy.
MedLine Citation:
PMID:  6875606     Owner:  NLM     Status:  MEDLINE    
Sialic acid is an important constituent of membrane-bound glycoproteins and glycolipids. It occurs linked to galactose at the surface of the membrane and is involved in, e.g., cation exchange, receptor function, maintenance of membrane polarity and intercellular interactions. In myotonic dystrophy there is evidence of an as yet basically undefined plasma-membrane abnormality. Considering the importance of sialic acid in membrane function, sialic acid as well as galactose concentrations were measured in erythrocyte membranes from 17 patients with myotonic dystrophy and compared to 17 matched healthy controls. There was a highly significant (P less than 0.0005) reduction of the sialic acid concentration in the patients, while no significant difference in galactose concentration was found. In 16 patients with limb-girdle and facioscapulohumeral dystrophy, sialic acid and galactose concentrations did not differ from matched controls. The possible importance of a reduced concentration of membrane-bound sialic acid in myotonic dystrophy is discussed in relation to previously reported biochemical membrane abnormalities in this disease.
H Stibler; O Sydow
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of the neurological sciences     Volume:  59     ISSN:  0022-510X     ISO Abbreviation:  J. Neurol. Sci.     Publication Date:  1983 Jun 
Date Detail:
Created Date:  1983-09-23     Completed Date:  1983-09-23     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  0375403     Medline TA:  J Neurol Sci     Country:  NETHERLANDS    
Other Details:
Languages:  eng     Pagination:  389-99     Citation Subset:  IM    
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MeSH Terms
Erythrocyte Membrane / analysis*
Erythrocytes / analysis*,  pathology
Galactose / analysis*
Middle Aged
Muscular Dystrophies / metabolism*
Myotonia Congenita / metabolism
Myotonic Dystrophy / metabolism*
Sialic Acids / analysis*
Reg. No./Substance:
0/Sialic Acids; 26566-61-0/Galactose

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

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