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

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16

作者:

JL HuestonKA Suprenant

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摘要:

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. Developmental Dynamics, 2009. 2009 Wiley‐Liss, Inc.

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DOI:

10.1002/dvdy.22007

年份:

2009

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