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Dev Dyn. 2009 Aug;238(8):1878-86. doi: 10.1002/dvdy.22007.

Loss of dystrophin and the microtubule-binding protein ELP-1 causes progressive paralysis and death of adult C. elegans.

Author information

1
Department of Molecular Biosciences, University of Kansas, Lawrence, Kansas 66045-7534, USA.

Abstract

EMAP-like proteins (ELPs) are conserved microtubule-binding proteins that function during cell division and in the behavior of post-mitotic cells. In Caenorhabditis elegans, ELP-1 is broadly expressed in many cells and tissues including the touch receptor neurons and body wall muscle. Within muscle, ELP-1 is associated with a microtubule network that is closely opposed to the integrin-based adhesion sites called dense bodies. To examine ELP-1 function, we utilized an elp-1 RNA interference assay and screened for synthetic interactions with mutated adhesion site proteins. We reveal a synthetic lethal relationship between ELP-1 and the dystrophin-like protein, DYS-1. Reduction of ELP-1 in a dystrophin [dys-1(cx18)] mutant results in adult animals with motility defects, splayed and hypercontracted muscle with altered cholinergic signaling. Worms fill with vesicles, become flaccid, and die. We conclude that ELP-1 is a genetic modifier of a C. elegans model of muscular dystrophy.

PMID:
19582871
PMCID:
PMC2942758
DOI:
10.1002/dvdy.22007
[Indexed for MEDLINE]
Free PMC Article

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