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Gene Review:

Flnc - filamin C, gamma

Mus musculus

Synonyms: 1110055E19Rik, ABP-280, ABP-280-like protein, ABP-L, ABPL, ...

Goetsch, S.C. et al., Beatham, J. et al., Lavu, S. et al., Patel, G. et al., Lypowy, J. et al., et al.

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Disease relevance of Flnc

These results suggest that KY is an intrinsic part of the protein networks underlying the molecular mechanism of several limb-girdle muscular dystrophies, particularly those where interactions between filamin C and disease causing proteins have been shown [1].
Filamin C interacts with the muscular dystrophy KY protein and is abnormally distributed in mouse KY deficient muscle fibres [1].

High impact information on Flnc

Second, a mouse model carrying a deletion of last 8 exons of Flnc was developed [2].
An alliance between Ras GTPase-activating protein, filamin C, and Ras GTPase-activating protein SH3 domain-binding protein regulates myocyte growth [3].
Additionally, tumor cells containing rearrangements in the myb locus, such as the ABPL tumors or NFS60 tumor cell line have recently been shown to display a heterogeneity of structure [4].
Structural organization and nucleotide sequence of mouse c-myb oncogene: activation in ABPL tumors is due to viral integration in an intron which results in the deletion of the 5' coding sequences [5].
Myogenic progenitor cells express filamin C in developing and regenerating skeletal muscle [6].

Biological context of Flnc

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 [6].
These results support the paradigm that the regulatory mechanisms of muscle regeneration largely recapitulate the fundamental events observed during muscle development and that filamin C may function in signal transduction or cellular migration of the myogenic progenitor cell population [6].

Anatomical context of Flnc

This expression pattern was further supported using RT-PCR analysis of filamin C expression in differentiating C2C12 myotubes [6].

Analytical, diagnostic and therapeutic context of Flnc

Using in situ hybridization, we observed filamin C to be restricted to mesodermal lineages including the developing heart and skeletal muscle during embryogenesis [6].

References

Filamin C interacts with the muscular dystrophy KY protein and is abnormally distributed in mouse KY deficient muscle fibres. Beatham, J., Romero, R., Townsend, S.K., Hacker, T., van der Ven, P.F., Blanco, G. Hum. Mol. Genet. (2004) [Pubmed]
Loss of FilaminC (FLNc) results in severe defects in myogenesis and myotube structure. Dalkilic, I., Schienda, J., Thompson, T.G., Kunkel, L.M. Mol. Cell. Biol. (2006) [Pubmed]
An alliance between Ras GTPase-activating protein, filamin C, and Ras GTPase-activating protein SH3 domain-binding protein regulates myocyte growth. Lypowy, J., Chen, I.Y., Abdellatif, M. J. Biol. Chem. (2005) [Pubmed]
Transcriptional activation potential of normal and tumor-associated myb isoforms does not correlate with their ability to block GCSF-induced terminal differentiation of murine myeloid precursor cells. Patel, G., Tantravahi, R., Oh, I.H., Reddy, E.P. Oncogene (1996) [Pubmed]
Structural organization and nucleotide sequence of mouse c-myb oncogene: activation in ABPL tumors is due to viral integration in an intron which results in the deletion of the 5' coding sequences. Lavu, S., Reddy, E.P. Nucleic Acids Res. (1986) [Pubmed]
Myogenic progenitor cells express filamin C in developing and regenerating skeletal muscle. Goetsch, S.C., Martin, C.M., Embree, L.J., Garry, D.J. Stem cells and development. (2005) [Pubmed]

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