Genomes and Genes
Gene Symbol: Flnc
Description: filamin C, gamma
Alias: 1110055E19Rik, ABP-280, ABPL, Fln2, filamin-C, ABP-280-like protein, actin-binding protein 280, actin-binding-like protein, filamin-2
- Bang M. Animal Models of Congenital Cardiomyopathies Associated With Mutations in Z-Line Proteins. J Cell Physiol. 2017;232:38-52 pubmed publisher..Pros and cons of mouse models will be discussed and a future outlook will be given. J. Cell. Physiol. 232: 38-52, 2017. © 2016 Wiley Periodicals, Inc. ..
- Zhang M, Liu J, Cheng A, Deyoung S, Saltiel A. Identification of CAP as a costameric protein that interacts with filamin C. Mol Biol Cell. 2007;18:4731-40 pubmed..Using both yeast two-hybrid and proteomic approaches, we identified the sarcomeric protein filamin C (FLNc) as a binding partner for CAP...
- Zhou X, Tian F, Sandzén J, Cao R, Flaberg E, Szekely L, et al. Filamin B deficiency in mice results in skeletal malformations and impaired microvascular development. Proc Natl Acad Sci U S A. 2007;104:3919-24 pubmed..These mice died or had to be euthanized before 4 weeks of age. Thus, the phenotypes of Flnb-deficient mice closely resemble those of human skeletal disorders with mutations in FLNB. ..
- Sarkisian M, Bartley C, Chi H, Nakamura F, Hashimoto Torii K, Torii M, et al. MEKK4 signaling regulates filamin expression and neuronal migration. Neuron. 2006;52:789-801 pubmed..Collectively, our results demonstrate a link between MEKK4 and Fln-A that impacts neuronal migration initiation and provides insight into the pathogenesis of human PVH. ..
- Otten J, van der Ven P, Vakeel P, Eulitz S, Kirfel G, Brandau O, et al. Complete loss of murine Xin results in a mild cardiac phenotype with altered distribution of intercalated discs. Cardiovasc Res. 2010;85:739-50 pubmed publisher..Furthermore, it indicates that XinC may be involved in the development of human cardiac hypertrophy. ..
- Dalkilic I, Schienda J, Thompson T, Kunkel L. Loss of FilaminC (FLNc) results in severe defects in myogenesis and myotube structure. Mol Cell Biol. 2006;26:6522-34 pubmedFilaminC (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...
- Molt S, Bührdel J, Yakovlev S, Schein P, Orfanos Z, Kirfel G, et al. Aciculin interacts with filamin C and Xin and is essential for myofibril assembly, remodeling and maintenance. J Cell Sci. 2014;127:3578-92 pubmed publisherb>Filamin C (FLNc) and Xin actin-binding repeat-containing proteins (XIRPs) are multi-adaptor proteins that are mainly expressed in cardiac and skeletal muscles and which play important roles in the assembly and repair of myofibrils and ..
- Goetsch S, Martin C, Embree L, Garry D. Myogenic progenitor cells express filamin C in developing and regenerating skeletal muscle. Stem Cells Dev. 2005;14:181-7 pubmed..To enhance our understanding of this cell population, we examined the temporal-spatial expression pattern for filamin C during murine embryogenesis, adult muscle regeneration and in selected myopathic models of human disease...
- Chevessier F, Schuld J, Orfanos Z, Plank A, Wolf L, Maerkens A, et al. Myofibrillar instability exacerbated by acute exercise in filaminopathy. Hum Mol Genet. 2015;24:7207-20 pubmed publisherb>Filamin C (FLNC) mutations in humans cause myofibrillar myopathy (MFM) and cardiomyopathy, characterized by protein aggregation and myofibrillar degeneration...
- Beatham J, Romero R, Townsend S, Hacker T, van der Ven P, Blanco G. Filamin C interacts with the muscular dystrophy KY protein and is abnormally distributed in mouse KY deficient muscle fibres. Hum Mol Genet. 2004;13:2863-74 pubmed..Here, we show that KY interacts with several sarcomeric cytoskeletal proteins including, amongst others, filamin C and the slow isoform of the myosin-binding protein C...
- Leber Y, Ruparelia A, Kirfel G, van der Ven P, Hoffmann B, Merkel R, et al. Filamin C is a highly dynamic protein associated with fast repair of myofibrillar microdamage. Hum Mol Genet. 2016;25:2776-2788 pubmedb>Filamin c (FLNc) is a large dimeric actin-binding protein located at premyofibrils, myofibrillar Z-discs and myofibrillar attachment sites of striated muscle cells, where it is involved in mechanical stabilization, mechanosensation and ..
- Cohen S, Brault J, Gygi S, Glass D, Valenzuela D, Gartner C, et al. During muscle atrophy, thick, but not thin, filament components are degraded by MuRF1-dependent ubiquitylation. J Cell Biol. 2009;185:1083-95 pubmed publisher..Because these proteins stabilize the thick filament, their selective ubiquitylation may facilitate thick filament disassembly. However, the thin filament components decreased by a mechanism not requiring MuRF1...
- Lu S, Carroll S, Herrera A, Ozanne B, Horowits R. New N-RAP-binding partners alpha-actinin, filamin and Krp1 detected by yeast two-hybrid screening: implications for myofibril assembly. J Cell Sci. 2003;116:2169-78 pubmed..The results suggest that sequential recruitment of N-RAP binding partners may serve an important role during myofibril assembly. ..
- Juo L, Liao W, Shih Y, Yang B, Liu A, Yan Y. HSPB7 interacts with dimerized FLNC and its absence results in progressive myopathy in skeletal muscles. J Cell Sci. 2016;129:1661-70 pubmed publisher..We identified dimerized filamin C (FLNC) as an interacting partner of HSPB7...