کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
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2037833 | 1072339 | 2008 | 12 صفحه PDF | دانلود رایگان |
![عکس صفحه اول مقاله: An Ankyrin-Based Mechanism for Functional Organization of Dystrophin and Dystroglycan An Ankyrin-Based Mechanism for Functional Organization of Dystrophin and Dystroglycan](/preview/png/2037833.png)
Summaryβ-dystroglycan (DG) and the dystrophin-glycoprotein complex (DGC) are localized at costameres and neuromuscular junctions in the sarcolemma of skeletal muscle. We present evidence for an ankyrin-based mechanism for sarcolemmal localization of dystrophin and β-DG. Dystrophin binds ankyrin-B and ankyrin-G, while β-DG binds ankyrin-G. Dystrophin and β-DG require ankyrin-G for retention at costameres but not delivery to the sarcolemma. Dystrophin and β-DG remain intracellular in ankyrin-B-depleted muscle, where β-DG accumulates in a juxta-TGN compartment. The neuromuscular junction requires ankyrin-B for localization of dystrophin/utrophin and β-DG and for maintenance of its postnatal morphology. A Becker muscular dystrophy mutation reduces ankyrin binding and impairs sarcolemmal localization of dystrophin-Dp71. Ankyrin-B also binds to dynactin-4, a dynactin subunit. Dynactin-4 and a subset of microtubules disappear from sarcolemmal sites in ankyrin-B-depleted muscle. Ankyrin-B thus is an adaptor required for sarcolemmal localization of dystrophin, as well as dynactin-4.
Journal: - Volume 135, Issue 7, 26 December 2008, Pages 1189–1200