Article
The earliest pathologic alterations in dysferlinopathy.
Neurology - 12 Jun 2001
Selcen D, Stilling G, Engel A G
Abstract excerpt
BACKGROUND: Dysferlinopathies are associated with proximal or distal muscular dystrophy. Dysferlin immunolocalizes to the muscle fiber periphery but does not associate with the dystrophin--glycoprotein complex; its function in humans, and the mechanism by which it causes muscle fiber injury, are not known. The authors therefore searched for pathogenetic clues by examining early abnormalities in nonnecrotic muscle...
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