The browser you are using is not supported by this website. All versions of Internet Explorer are no longer supported, either by us or Microsoft (read more here: https://www.microsoft.com/en-us/microsoft-365/windows/end-of-ie-support).

Please use a modern browser to fully experience our website, such as the newest versions of Edge, Chrome, Firefox or Safari etc.

Default user image.

Sebastian Kalamajski

Assistant researcher

Default user image.

Cib2 binds integrin a7Bb1D and is reduced in laminin a2 chain deficient muscular dystrophy

Author

  • Mattias Häger
  • Bigotti Maria Giulia
  • Renata Gustafsson
  • Virginie Carmignac
  • Johan K Holmberg
  • Allamand Valerie
  • Mikael Åkerlund
  • Sebastian Kalamajski
  • Brancaccio Andrea
  • Mayer Ulrike
  • Madeleine Durbeej-Hjalt

Summary, in English

Mutations in the gene encoding laminin alpha 2 chain cause congenital muscular dystrophy type 1A. In skeletal muscle, laminin alpha 2 chain binds at least two receptor complexes: the dystrophin-glycoprotein complex and integrin alpha 7 beta 1. To gain insight into the molecular mechanisms underlying this disorder, we performed gene expression profiling of laminin alpha 2 chain-deficient mouse limb muscle. One of the down-regulated genes encodes a protein called Cib2 (calcium-and integrin-binding protein 2) whose expression and function is unknown. However, the closely related Cib1 has been reported to bind integrin alpha IIb and may be involved in outside-in-signaling in platelets. Since Cib2 might be a novel integrin alpha 7 beta 1-binding protein in muscle, we have studied Cib2 expression in the developing and adult mouse. Cib2 mRNA is mainly expressed in the developing central nervous system and in developing and adult skeletal muscle. In skeletal muscle, Cib2 colocalizes with the integrin alpha 7B subunit at the sarcolemma and at the neuromuscular and myotendinous junctions. Finally, we demonstrate that Cib2 is a calcium-binding protein that interacts with integrin alpha 7B beta 1D. Thus, our data suggest a role for Cib2 as a cytoplasmic effector of integrin alpha 7B beta 1D signaling in skeletal muscle.

Department/s

  • Muscle Biology
  • Department of Experimental Medical Science

Publishing year

2008

Language

English

Pages

24760-24769

Publication/Series

Journal of Biological Chemistry

Volume

283

Issue

36

Document type

Journal article

Publisher

ASBMB

Topic

  • Cell and Molecular Biology

Status

Published

Research group

  • Muscle Biology

ISBN/ISSN/Other

  • ISSN: 1083-351X