|  Help  |  About  |  Contact Us

Publication : Loss of FilaminC (FLNc) results in severe defects in myogenesis and myotube structure.

First Author  Dalkilic I Year  2006
Journal  Mol Cell Biol Volume  26
Issue  17 Pages  6522-34
PubMed ID  16914736 Mgi Jnum  J:112170
Mgi Id  MGI:3655755 Doi  10.1128/MCB.00243-06
Citation  Dalkilic I, et al. (2006) Loss of FilaminC (FLNc) results in severe defects in myogenesis and myotube structure. Mol Cell Biol 26(17):6522-34
abstractText  FilaminC (FLNc) is the muscle-specific member of a family of actin binding proteins. Although it interacts with many proteins involved in muscular dystrophies, its unique role in muscle is poorly understood. To address this, two models were developed. First, FLNc expression was stably reduced in C2C12 myoblasts by RNA interference. While these cells start differentiation normally, they display defects in differentiation and fusion ability and ultimately form multinucleated 'myoballs' rather than maintain elongated morphology. Second, a mouse model carrying a deletion of last 8 exons of Flnc was developed. FLNc-deficient mice die shortly after birth, due to respiratory failure, and have severely reduced birth weights, with fewer muscle fibers and primary myotubes, indicating defects in primary myogenesis. They exhibit variation in fiber size, fibers with centrally located nuclei, and some rounded fibers resembling the in vitro phenotype. The similarity of the phenotype of FLNc-deficient mice to the filamin-interacting TRIO null mice was further confirmed by comparing FLNc-deficient C2C12 cells to TRIO-deficient cells. These data provide the first evidence that FLNc has a crucial role in muscle development and maintenance of muscle structural integrity and suggest the presence of a TRIO-FLNc-dependent pathway in maintaining proper myotube structure.
Quick Links:
Quick Links:


Publication --> Expression annotations



7 Bio Entities

Trail: Publication

0 Expression