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Confocal analysis of the dystrophin protein complex in muscular dystrophy
Author(s) -
Draviam Romesh,
Billington Lynn,
Senchak Andy,
Hoffman Eric P.,
Watkins Simon C.
Publication year - 2001
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/1097-4598(200102)24:2<262::aid-mus120>3.0.co;2-3
Subject(s) - dystrophin , immunostaining , muscular dystrophy , biology , pathology , extracellular matrix , utrophin , sarcolemma , microbiology and biotechnology , itga7 , myocyte , immunohistochemistry , medicine , genetics
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 β‐sarcoglycan was expressed appropriately at the sarcolemma of γ‐sarcoglycan–deficient patients, intracellular labeling of β‐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. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 262–272, 2001