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Article

Disrupted myosin cross-bridge cycling kinetics triggers muscle weakness in nebulin-related myopathy.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Jun 2011

Ochala Julien, Lehtokari Vilma-Lotta, Iwamoto Hiroyuki, Li Meishan, Feng Han-Zhong, Jin Jian-Ping, Yagi Naoto, Wallgren-Pettersson Carina, Pénisson-Besnier Isabelle, Larsson Lars

Abstract excerpt

Nebulin is a giant protein expressed at high levels in skeletal muscle. Mutations in the nebulin gene (NEB) lead to muscle weakness and various congenital myopathies. Despite increasing clinical and scientific interest, the pathogenesis of weakness remains unknown. The present study, therefore, aims at unraveling the underlying molecular mechanisms. Hence, we recorded and analyzed the mechanics as well as the...

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Share to Myopathies, Nemaline

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Source

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Identifiers

PMID: 21350120
DOI: 10.1096/fj.10-176727
PMCID: PMC3219215

Genes

NEB

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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