Ontology highlight
ABSTRACT:
SUBMITTER: Fernandes I
PROVIDER: S-EPMC4137052 | biostudies-literature | 2014 Aug
REPOSITORIES: biostudies-literature
Fernandes Isabelle I Schöck Frieder F
The Journal of cell biology 20140811 4
Mutations in nebulin, a giant muscle protein with 185 actin-binding nebulin repeats, are the major cause of nemaline myopathy in humans. Nebulin sets actin thin filament length in sarcomeres, potentially by stabilizing thin filaments in the I-band, where nebulin and thin filaments coalign. However, the precise role of nebulin in setting thin filament length and its other functions in regulating power output are unknown. Here, we show that Lasp, the only member of the nebulin family in Drosophila ...[more]