Immunogen
Synthesized peptide derived from human FLNC AA range: 335-385
Specificity
This antibody detects endogenous levels of FLNC at Human/Mouse
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
IHC-p 1：50-200. IF 1:50-200
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This gene encodes one of three related filamin genes, specifically gamma filamin. These filamin proteins crosslink actin filaments into orthogonal networks in cortical cytoplasm and participate in the anchoring of membrane proteins for the actin cytoskeleton. Three functional domains exist in filamin: an N-terminal filamentous actin-binding domain, a C-terminal self-association domain, and a membrane glycoprotein-binding domain. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008],
Function
developmental stage:Expressed in both differentiating and adult muscles.,disease:Defects in FLNC are the cause of autosomal dominant filaminopathy [MIM:609524, 601419]. Myofibrillar myopathy (MFM) is a neuromuscular disorder, usually with an adult onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations. Autosomal dominant filaminopathy is a form of MFM characterized by morphological features of MFM and clinical features of a limb-girdle myopathy. A heterozygous nonsense mutation which segregates with the disease, has been identified in the FLNC gene.,domain:Comprised of a NH2-terminal actin-binding domain, 24 internally homologous repeats and two hinge regions. Repeat 24 and the second hinge domain are important for dimer formation.,domain:The filamin 20 repeat mediates interaction with XIRP1.,domain:The intradomain insert is specific to F
Fields
>>MAPK signaling pathway;>>Focal adhesion;>>Salmonella infection;>>Proteoglycans in cancer
Human protein sequence Database
Q14315
Mouse protein sequence database
Q8VHX6
Rat protein sequence database
Cellular localization
Cytoplasm . Membrane ; Peripheral membrane protein . Cytoplasm, cytoskeleton . Cytoplasm, myofibril, sarcomere, Z line . 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.
Tissue expression
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.
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