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An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan.

Publication ,  Journal Article
Ayalon, G; Davis, JQ; Scotland, PB; Bennett, V
Published in: Cell
December 26, 2008

beta-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 beta-DG. Dystrophin binds ankyrin-B and ankyrin-G, while beta-DG binds ankyrin-G. Dystrophin and beta-DG require ankyrin-G for retention at costameres but not delivery to the sarcolemma. Dystrophin and beta-DG remain intracellular in ankyrin-B-depleted muscle, where beta-DG accumulates in a juxta-TGN compartment. The neuromuscular junction requires ankyrin-B for localization of dystrophin/utrophin and beta-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.

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Published In

Cell

DOI

EISSN

1097-4172

Publication Date

December 26, 2008

Volume

135

Issue

7

Start / End Page

1189 / 1200

Location

United States

Related Subject Headings

  • Sequence Alignment
  • Sarcolemma
  • Neuromuscular Junction
  • Muscular Dystrophy, Duchenne
  • Muscle, Skeletal
  • Molecular Sequence Data
  • Microtubules
  • Microtubule-Associated Proteins
  • Mice
  • Dystrophin
 

Citation

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Ayalon, G., Davis, J. Q., Scotland, P. B., & Bennett, V. (2008). An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan. Cell, 135(7), 1189–1200. https://doi.org/10.1016/j.cell.2008.10.018
Ayalon, Gai, Jonathan Q. Davis, Paula B. Scotland, and Vann Bennett. “An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan.Cell 135, no. 7 (December 26, 2008): 1189–1200. https://doi.org/10.1016/j.cell.2008.10.018.
Ayalon G, Davis JQ, Scotland PB, Bennett V. An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan. Cell. 2008 Dec 26;135(7):1189–200.
Ayalon, Gai, et al. “An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan.Cell, vol. 135, no. 7, Dec. 2008, pp. 1189–200. Pubmed, doi:10.1016/j.cell.2008.10.018.
Ayalon G, Davis JQ, Scotland PB, Bennett V. An ankyrin-based mechanism for functional organization of dystrophin and dystroglycan. Cell. 2008 Dec 26;135(7):1189–1200.
Journal cover image

Published In

Cell

DOI

EISSN

1097-4172

Publication Date

December 26, 2008

Volume

135

Issue

7

Start / End Page

1189 / 1200

Location

United States

Related Subject Headings

  • Sequence Alignment
  • Sarcolemma
  • Neuromuscular Junction
  • Muscular Dystrophy, Duchenne
  • Muscle, Skeletal
  • Molecular Sequence Data
  • Microtubules
  • Microtubule-Associated Proteins
  • Mice
  • Dystrophin