Laminin alpha1 chain mediated reduction of laminin alpha2 chain deficient muscular dystrophy involves integrin alpha7beta1 and dystroglycan.

Abstract:

:Transgenically introduced laminin (LN) alpha1 chain prevents muscular dystrophy in LNalpha2 chain deficient mice. We now report increased integrin alpha7Bbeta1D synthesis in dystrophic LNalpha2 chain deficient muscle. Yet, immunofluorescence demonstrated a reduced expression of integrin alpha7B subunit at the sarcolemma. Transgenic expression of LNalpha1 chain reconstituted integrin alpha7B at the sarcolemma. Expression of alpha- and beta-dystroglycan is enhanced in LNalpha2 chain deficient muscle and normalized by transgenic expression of LNalpha1 chain. We suggest that LNalpha1 chain in part ameliorates the development of LNalpha2 chain deficient muscular dystrophy by retaining the binding sites for integrin alpha7Bbeta1D and alpha-dystroglycan, respectively.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

Gawlik KI,Mayer U,Blomberg K,Sonnenberg A,Ekblom P,Durbeej M

doi

10.1016/j.febslet.2006.02.027

keywords:

subject

Has Abstract

pub_date

2006-03-20 00:00:00

pages

1759-65

issue

7

eissn

0014-5793

issn

1873-3468

pii

S0014-5793(06)00225-0

journal_volume

580

pub_type

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