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The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain.
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MedLine Citation:
PMID:  1400579     Owner:  NLM     Status:  MEDLINE    
Chromosome 6-encoded dystrophin-related-protein (DRP) shows significant structural similarities to dystrophin at the carboxyl terminus, though the two proteins are encoded on different chromosomes. Both DRP and dystrophin are expressed in muscle and brain and show some similarity in their subcellular localization. For example, in skeletal muscle both are expressed at neuromuscular and myotendinous junctions. However, while dystrophin is absent or severely reduced in Duchenne/Becker muscular dystrophy, DRP continues to be expressed. Within the brain, dystrophin is enriched at the postsynaptic regions of specific subsets of neurons, while the distribution of DRP is yet to be described. In this study we demonstrate a distinct though highly specific pattern of distribution of DRP in the brain. DRP is enriched in the choroid plexus, pia mater, intracerebral vasculature, and ependymal lining. Within the parenchyma proper, DRP is located at the inner plasma face of astrocytic foot processes at the abluminal aspect of the blood-brain barrier. The distribution of DRP is conserved across a large evolutionary distance, from mammals to elasmobranchs, suggesting that DRP may play a role in the maintenance of regional specializations in the brain.
T S Khurana; S C Watkins; L M Kunkel
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  The Journal of cell biology     Volume:  119     ISSN:  0021-9525     ISO Abbreviation:  J. Cell Biol.     Publication Date:  1992 Oct 
Date Detail:
Created Date:  1992-11-19     Completed Date:  1992-11-19     Revised Date:  2010-09-07    
Medline Journal Info:
Nlm Unique ID:  0375356     Medline TA:  J Cell Biol     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  357-66     Citation Subset:  IM    
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
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MeSH Terms
Astrocytes / chemistry
Blood-Brain Barrier
Brain Chemistry*
Cells, Cultured
Choroid Plexus / chemistry
Cross Reactions
Cytoskeletal Proteins / isolation & purification*
Disease Models, Animal
Fluorescent Antibody Technique
Membrane Proteins*
Mice, Mutant Strains
Muscular Dystrophy, Animal / pathology*
Skates (Fish)
Species Specificity
Reg. No./Substance:
0/Cytoskeletal Proteins; 0/Membrane Proteins; 0/Utrn protein, mouse; 0/Utrophin

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

Full Text
Journal Information
Journal ID (nlm-ta): J Cell Biol
Journal ID (publisher-id): J. Cell Biol.
ISSN: 0021-9525
ISSN: 1540-8140
Publisher: The Rockefeller University Press
Article Information
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Print publication date: Day: 2 Month: 10 Year: 1992
Volume: 119 Issue: 2
First Page: 357 Last Page: 366
ID: 2289652
Publisher Id: 93016250
PubMed Id: 1400579

The subcellular distribution of chromosome 6-encoded dystrophin-related protein in the brain

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