Flnc

Summary

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
Species: mouse
Products:     Flnc

Top Publications

  1. Gariboldi M, Maestrini E, Canzian F, Manenti G, De Gregorio L, Rivella S, et al. Comparative mapping of the actin-binding protein 280 genes in human and mouse. Genomics. 1994;21:428-30 pubmed
    ..ABP-280 or filamin (FLN1) is present in the cytoskeleton of many cell types, whereas expression of FLN2 is limited to skeletal muscle and heart...
  2. Chiang W, Greaser M, Lyons G. Filamin isogene expression during mouse myogenesis. Dev Dyn. 2000;217:99-108 pubmed
    ..This is the first study of filamin gene expression at the early stages of skeletal muscle development. Dev Dyn 2000;217:99-108. ..
  3. van der Flier A, Kuikman I, Kramer D, Geerts D, Kreft M, Takafuta T, et al. Different splice variants of filamin-B affect myogenesis, subcellular distribution, and determine binding to integrin [beta] subunits. J Cell Biol. 2002;156:361-76 pubmed
    ..These findings suggest that specific combinations of filamin mRNA splicing events modulate the organization of the actin cytoskeleton and the binding affinity for integrins. ..
  4. Janssens J, Philtjens S, Kleinberger G, Van Mossevelde S, van der Zee J, Cacace R, et al. Investigating the role of filamin C in Belgian patients with frontotemporal dementia linked to GRN deficiency in FTLD-TDP brains. Acta Neuropathol Commun. 2015;3:68 pubmed publisher
    ..Loss of TDP-43 in zebrafish engenders a severe muscle and vascular phenotype with a concomitant elevation of filamin C (FLNC) levels, an observation confirmed in the frontal cortex of FTLD-TDP patients...
  5. Mastrototaro G, Liang X, Li X, Carullo P, Piroddi N, Tesi C, et al. Nebulette knockout mice have normal cardiac function, but show Z-line widening and up-regulation of cardiac stress markers. Cardiovasc Res. 2015;107:216-25 pubmed publisher
    ..These results suggest that the nebulette disease causing mutations have dominant gain-of-function effects. ..
  6. Gurniak C, Chevessier F, Jokwitz M, Jönsson F, Perlas E, Richter H, et al. Severe protein aggregate myopathy in a knockout mouse model points to an essential role of cofilin2 in sarcomeric actin exchange and muscle maintenance. Eur J Cell Biol. 2014;93:252-66 pubmed publisher
    ..Levels of smooth muscle ?-actin were increased and remained high in developing muscles, suggesting that cofilin2 plays a crucial role during the exchange of ?-actin isoforms during the early postnatal remodeling of the sarcomere. ..
  7. Baker J, Riley G, Romero M, Haynes A, Hilton H, Simon M, et al. Identification of a Z-band associated protein complex involving KY, FLNC and IGFN1. Exp Cell Res. 2010;316:1856-70 pubmed publisher
    ..A yeast two-hybrid screen identified the Z-band protein filamin C (FLNC) as an interacting partner...
  8. Fielitz J, van Rooij E, Spencer J, Shelton J, Latif S, van der Nagel R, et al. Loss of muscle-specific RING-finger 3 predisposes the heart to cardiac rupture after myocardial infarction. Proc Natl Acad Sci U S A. 2007;104:4377-82 pubmed
    ..These findings reveal an important role of MuRF3 in maintaining cardiac integrity and function after acute myocardial infarction and suggest that turnover of FHL2 and gamma-filamin contributes to this cardioprotective function of MuRF3. ..
  9. Liao W, Juo L, Shih Y, Chen Y, Yan Y. HSPB7 prevents cardiac conduction system defect through maintaining intercalated disc integrity. PLoS Genet. 2017;13:e1006984 pubmed publisher
    ..Furthermore, filamin C, the interaction protein of HSPB7, was upregulated and aggregated in HSPB7 mutant cardiomyocytes...

More Information

Publications24

  1. 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. ..
  2. 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...
  3. 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. ..
  4. 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. ..
  5. 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. ..
  6. 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 pubmed
    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...
  7. 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 publisher
    b>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 ..
  8. 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...
  9. 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 publisher
    b>Filamin C (FLNC) mutations in humans cause myofibrillar myopathy (MFM) and cardiomyopathy, characterized by protein aggregation and myofibrillar degeneration...
  10. 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...
  11. 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 pubmed
    b>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 ..
  12. 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...
  13. 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. ..
  14. Lu Y, Adegoke O, Nepveu A, Nakayama K, Bedard N, Cheng D, et al. USP19 deubiquitinating enzyme supports cell proliferation by stabilizing KPC1, a ubiquitin ligase for p27Kip1. Mol Cell Biol. 2009;29:547-58 pubmed publisher
  15. 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...