Article
KLHL41 stabilizes skeletal muscle sarcomeres by nonproteolytic ubiquitination.
eLife - 9 Aug 2017
Ramirez-Martinez Andres, Cenik Bercin Kutluk, Bezprozvannaya Svetlana, Chen Beibei, Bassel-Duby Rhonda, Liu Ning, Olson Eric N
Abstract excerpt
Maintenance of muscle function requires assembly of contractile proteins into highly organized sarcomeres. Mutations in Kelch-like protein 41 (KLHL41) cause nemaline myopathy, a fatal muscle disorder associated with sarcomere disarray. We generated KLHL41 mutant mice, which display lethal disruption of sarcomeres and aberrant expression of muscle structural and contractile proteins, mimicking the hallmarks of the...
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