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PMID: 1400579 Published · ppublish English 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.

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

The Journal of cell biology ·Vol. 119 ·No. 2 ·1992-10-00 ·Pages 357-66

Khurana TS, Watkins SC, Kunkel LM

Abstract

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.

Related Genes
mdx
MeSH Terms
Animals Astrocytes/chemistry Blood-Brain Barrier Brain Chemistry Cells, Cultured Choroid Plexus/chemistry Cross Reactions Cytoskeletal Proteins/isolation & purification Disease Models, Animal Fluorescent Antibody Technique Humans Membrane Proteins Mice Mice, Mutant Strains Muscular Dystrophy, Animal/pathology Skates, Fish Species Specificity Utrophin
Chemicals
Cytoskeletal Proteins Membrane Proteins Utrn protein, mouse Utrophin
Authors & Affiliations
3 authors, click to expand affiliations / ORCID
Khurana T S
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Watkins S C
Kunkel L M
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Article Info
Journal
The Journal of cell biology
Abbr.
J Cell Biol
ISSN
0021-9525
DOI
10.1083/jcb.119.2.357
Published
1992-10-00
Pages
357-66
Language
English
Region
United States
NLM ID
0375356
PMCID
PMC2289652
Subset
IM
External Links
PubMed source DOI: 10.1083/jcb.119.2.357 PMC Full Text View journal details
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