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Regulation and functional significance of utrophin expression at the mammalian neuromuscular synapse
Author(s) -
Gramolini Anthony O.,
Wu Jun,
Jasmin Bernard J.
Publication year - 2000
Publication title -
microscopy research and technique
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.536
H-Index - 118
eISSN - 1097-0029
pISSN - 1059-910X
DOI - 10.1002/(sici)1097-0029(20000401)49:1<90::aid-jemt10>3.0.co;2-l
Subject(s) - utrophin , dystrophin , skeletal muscle , sarcolemma , microbiology and biotechnology , duchenne muscular dystrophy , neuromuscular junction , itga7 , actin , biology , muscular dystrophy , myocyte , chemistry , neuroscience , anatomy , genetics
Duchenne muscular dystrophy (DMD) is caused by the absence of full‐length dystrophin molecules in skeletal muscle fibers. In normal muscle, dystrophin is found along the length of the sarcolemma where it links the intracellular actin cytoskeleton to the extracellular matrix, via the dystrophin‐associated protein (DAP) complex. Several years ago, an autosomal homologue to dystrophin, termed utrophin, was identified and shown to be expressed in a variety of tissues, including skeletal muscle. However, in contrast to the localization of dystrophin in extrajunctional regions of muscle fibers, utrophin preferentially accumulates at the postsynaptic membrane of the neuromuscular junction in both normal and DMD adult muscle fibers. Since it has recently been suggested that the upregulation of utrophin might functionally compensate for the lack of dystrophin in DMD, considerable interest is now directed toward the elucidation of the various regulatory mechanisms presiding over expression of utrophin in normal and dystrophic skeletal muscle fibers. In this review, we discuss some of the most recent data relevant to our understanding of the impact of myogenic differentiation and innervation on the expression and localization of utrophin in skeletal muscle fibers. Microsc. Res. Tech. 49:90–100, 2000. © 2000 Wiley‐Liss, Inc.