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Titolo:
Assembly of the dystrophin-associated protein complex does not require thedystrophin COOH-terminal domain
Autore:
Crawford, GE; Faulkner, JA; Crosbie, RH; Campbell, KP; Froehner, SC; Chamberlain, JS;
Indirizzi:
Univ Michigan, Dept Human Genet, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 Human Genet, Ann Arbor, MI 48109 USA Univ Michigan, Mol & Cellular Biol Program, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 iol Program, Ann Arbor, MI 48109 USA Univ Michigan, Ctr Gene Therapy, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 ene Therapy, Ann Arbor, MI 48109 USA Univ Michigan, Dept Physiol, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 ept Physiol, Ann Arbor, MI 48109 USA Univ Iowa, Coll Med, Howard Hughes Med Inst, Dept Physiol & Biophys, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 ysiol & Biophys, Iowa City, IA 52242 USA Univ Iowa, Coll Med, Dept Neurol, Iowa City, IA 52242 USA Univ Iowa Iowa City IA USA 52242 ed, Dept Neurol, Iowa City, IA 52242 USA Univ N Carolina, Dept Cell & Mol Physiol, Chapel Hill, NC 27599 USA Univ NCarolina Chapel Hill NC USA 27599 ysiol, Chapel Hill, NC 27599 USA
Titolo Testata:
JOURNAL OF CELL BIOLOGY
fascicolo: 6, volume: 150, anno: 2000,
pagine: 1399 - 1409
SICI:
0021-9525(20000918)150:6<1399:AOTDPC>2.0.ZU;2-D
Fonte:
ISI
Lingua:
ENG
Soggetto:
DUCHENNE MUSCULAR-DYSTROPHY; NITRIC-OXIDE SYNTHASE; SARCOGLYCAN-DEFICIENT MICE; GLYCOPROTEIN-BINDING-SITE; SKELETAL-MUSCLE; MDX MICE; ALPHA-DYSTROBREVIN; POSTSYNAPTIC PROTEIN; BETA-DYSTROBREVIN; GAMMA-SARCOGLYCAN;
Keywords:
dystrophin; muscular dystrophy; syntrophin; dystrobrevin; mdx mice;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
82
Recensione:
Indirizzi per estratti:
Indirizzo: Chamberlain, JS Univ Michigan, Dept Human Genet, Ann Arbor, MI 48109 USA Univ Michigan Ann Arbor MI USA 48109 Arbor, MI 48109 USA
Citazione:
G.E. Crawford et al., "Assembly of the dystrophin-associated protein complex does not require thedystrophin COOH-terminal domain", J CELL BIOL, 150(6), 2000, pp. 1399-1409

Abstract

Dystrophin is a multidomain protein that links the actin cytoskeleton to laminin in the extracellular matrix through the dystrophin associated protein (DAP) complex. The COOH-terminal domain of dystrophin binds to two components of the DAP complex, syntrophin and dystrobrevin. To understand the role of syntrophin and dystrobrevin, we previously generated a series of transgenic mouse lines expressing dystrophins with deletions throughout the COOH-terminal domain. Each of these mice had normal muscle function and displayed normal localization of syntrophin and dystrobrevin, Since syntrophin anddystrobrevin bind to each other as well as to dystrophin, we have now generated a transgenic mouse deleted for the entire dystrophin COOH-terminal domain. Unexpectedly, this truncated dystrophin supported normal muscle function and assembly of the DAP complex. These results demonstrate that syntrophin and dystrobrevin functionally associate with the DAP complex in the absence of a di;ect link to dystrophin. We also observed that the DAP complexes in these different transgenic mouse strains were not identical. Instead, the DAP complexes contained varying ratios of syntrophin and dystrobrevin isoforms. These results suggest that alternative splicing of the dystrophin gene, which naturally generates COOH-terminal deletions in dystrophin, may function to regulate the isoform composition of the DAP complex.

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Documento generato il 04/12/20 alle ore 00:53:49