Confocal analysis of the dystrophin protein complex in muscular dystrophy.

Abstract:

:The dystrophin protein complex (DPC), composed of at least 10 proteins that associate with dystrophin, is critical for the maintenance of normal muscle fiber structure and physiology. In this study, we used immunohistochemistry and confocal microscopy to examine the relative abundance and distribution of several of these proteins in muscle biopsies taken from patients with various muscular dystrophies. The optical sectioning capability of confocal microscopy allowed us to comprehensively analyze the semiquantitative expression of components of the DPC. Alpha-sarcoglycan-deficient patients displayed a marked reduction in membrane immunostaining of the sarcoglycan complex. Gamma-sarcoglycan-deficient patients showed variable decreased immunostaining of the sarcoglycan complex proteins. When beta-sarcoglycan was expressed appropriately at the sarcolemma of gamma-sarcoglycan-deficient patients, intracellular labeling of beta-sarcoglycan was also present. Beta-sarcoglycan-deficient patients showed poor localization of extracellular matrix proteins in addition to a complete absence of the sarcoglycans. Merosin-deficient patients showed relatively normal immunostaining levels of all other members of the DPC. Finally, dystrophin-deficient patients showed little or no change in the expression of extracellular matrix proteins; however, some sarcoglycans were significantly decreased. These data allowed us to suggest unique fundamental interactions between the members of the DPC.

journal_name

Muscle Nerve

journal_title

Muscle & nerve

authors

Draviam R,Billington L,Senchak A,Hoffman EP,Watkins SC

doi

10.1002/1097-4598(200102)24:2<262::aid-mus120>3.0.

subject

Has Abstract

pub_date

2001-02-01 00:00:00

pages

262-72

issue

2

eissn

0148-639X

issn

1097-4598

pii

10.1002/1097-4598(200102)24:2<262::AID-MUS120>3.0.

journal_volume

24

pub_type

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