The Subcellular Distribution of Chromosome 6-encoded Dystrophin-related Protein in the Brain

Overview

Journal J Cell Biol

Specialty Cell Biology

Date 1992 Oct 1

PMID 1400579

Citations 18

Authors

T S Khurana

S C Watkins

L M Kunkel

Affiliations

Soon will be listed here.

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.

Citing Articles

Dystrophins, utrophins, and associated scaffolding complexes: role in mammalian brain and implications for therapeutic strategies.

Perronnet C, Vaillend C J Biomed Biotechnol. 2010; 2010:849426.

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Components of the basal lamina and dystrophin-dystroglycan complex in the neurointermediate lobe of rat pituitary gland: different localizations of beta-dystroglycan, dystrobrevins, alpha1-syntrophin, and aquaporin-4.

Pocsai K, Bagyura Z, Kalman M J Histochem Cytochem. 2010; 58(5):463-79.

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Differential expression of utrophin-A and -B promoters in the central nervous system (CNS) of normal and dystrophic mdx mice.

Baby S, Bogdanovich S, Willmann G, Basu U, Lozynska O, Khurana T Brain Pathol. 2009; 20(2):323-42.

PMID: 19486009 PMC: 8095546. DOI: 10.1111/j.1750-3639.2009.00275.x.

Dystrophin and utrophin isoforms are expressed in glia, but not neurons, of the avian parasympathetic ciliary ganglion.

Blitzblau R, Storer E, Jacob M Brain Res. 2008; 1218:21-34.

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ZZ domain of dystrophin and utrophin: topology and mapping of a beta-dystroglycan interaction site.

Hnia K, Zouiten D, Cantel S, Chazalette D, Hugon G, Fehrentz J Biochem J. 2006; 401(3):667-77.

PMID: 17009962 PMC: 1770854. DOI: 10.1042/BJ20061051.

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