Proteasome inhibitors reduce thrombospondin-1 release in human dysferlin-deficient myotubes
Abstract
Background Dysferlinopathies are a group of muscle disorders causing muscle weakness and absence or low levels of dysferlin, a type-II transmembrane protein and the causative gene of these dystrophies. Dysferlin is implicated in vesicle fusion, trafficking, and membrane repair. Muscle biopsy of patients with dysferlinopathy is characterized by the presence of inflammatory infiltrates. Studies in the muscle of both human and mouse models of...
Paper Details
Title
Proteasome inhibitors reduce thrombospondin-1 release in human dysferlin-deficient myotubes
Published Date
Nov 27, 2020
Volume
21
Issue
1
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