CGC Bibliography Paper 5521

Dystrophin and functionally related proteins in the nematode Caenorhabditis elegans.

Segalat L

Medline:
12206804
Citation:
Neuromuscular Disorders 12: S105-S109 2002
Type:
REVIEW
Genes:
dyb-1 dyc-1 dys-1 dys-2 dys-3 dys-4 dys-5 hlh-1
Abstract:
We investigated the function of dystrophin in the nematode Caenorhabditis elegans. Although nematodes and mammals diverged early in evolution, their muscles share many structural and molecular features, thus rendering C. elegans relevant as a model to study muscle function. Dystrophin, dystrobrevin, dystroglycans and several sarcoglycans have conserved homologues in the genome of C. elegans. The major strength of the model comes from its genetic tractability, which allows the quick and easy manipulation of gene expression, either to inactivate genes, or to create transgenic animals. Over the last 2 years, work on C. elegans dystrophin has led to the identification of a putative new member of the dystrophin-glycoprotein complex, and has brought additional data suggesting that dystrophin mutations affect ion