Recombinant Human Filamin-C (FLNC), Truncated

Uniprot Id

Q14315

Target Species

Human

Target Name

FLNC

Target Full Name

Filamin-C

Target Function

Muscle-specific filamin, which plays a central role in muscle cells, probably by functioning as a large actin-cross-linking protein. May be involved in reorganizing the actin cytoskeleton in response to signaling events, and may also display structural functions at the Z lines in muscle cells. Critical for normal myogenesis and for maintaining the structural integrity of the muscle fibers.

Target Involvement

Myopathy, myofibrillar, 5 (MFM5); Myopathy, distal, 4 (MPD4); Cardiomyopathy, familial hypertrophic 26 (CMH26); Cardiomyopathy, familial restrictive 5 (RCM5)

Target Subcellular Location

Cytoplasm. Membrane; Peripheral membrane protein. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line. Note=A small amount localizes at membranes. In striated muscle cells, it predominantly localizes in myofibrillar Z lines, while a minor fraction localizes with subsarcolemme. Targeting to developing and mature Z lines is mediated by the intradomain insert.

Target Protein Families

Filamin family

Target Tissue Specificity

Highly expressed in striated muscles. Weakly expressed in thyroid, fetal brain, fetal lung, retina, spinal cord and bone marrow. Not expressed in testis, pancreas, adrenal gland, placenta, liver and kidney.

Target Synonyms

FLNC; ABPL; FLN2Filamin-C; FLN-C; FLNc; ABP-280-like protein; ABP-L; Actin-binding-like protein; Filamin-2; Gamma-filamin