catalog number :
MBS2026155
products full name :
Polyclonal Antibody to Filamin C Gamma (FLNC)
products short name :
[Filamin C Gamma]
other names :
[filamin-C isoform b; Filamin-C; filamin-C; filamin C; ABP-280-like protein; ABP-L; Actin-binding-like protein; Filamin-2; Gamma-filamin]
products gene name :
[FLNC]
other gene names :
[FLNC; FLNC; ABPA; ABPL; FLN2; MFM5; MPD4; RCM5; CMH26; ABP-280; ABP280A; ABPL; FLN2; FLN-C; FLNc]
purity :
Antigen-specific affinity chromatography followed by Protein A affinity chromatography
form :
Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3, 50% glycerol.
concentration :
0.25mg/ml
storage stability :
Storage: Avoid repeated freeze/thaw cycles. Store at 4ºC for frequent use. Aliquot and store at -20ºC for 24 months. Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no
obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
tested application :
Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunoprecipitation (IP)
app notes :
Western blotting: 0.5-2ug/mL;. Immunohistochemistry: 5-20ug/mL;. Immunocytochemistry: 5-20ug/mL;. Optimal working dilutions must be determined by end user.
image1 heading :
Western Blot (WB)
image2 heading :
Immunohistochemistry (IHC)
image3 heading :
Immunohistochemistry (IHC)
other info1 :
Organism Species: Homo sapiens (Human). Source: Polyclonal antibody preparation. Traits: Liquid. Immunogen: Recombinant FLNC (Glu17~Lys259 (Accession # Q14315)) expressed in E.coli ( MBS2030652 )
ncbi acc num :
NP_001120959.1
ncbi gb acc num :
NM_001127487.1
ncbi pathways :
Cell Junction Organization Pathway (1270232); Cell-Cell Communication Pathway (1270231); Cell-extracellular Matrix Interactions Pathway (1270237); Focal Adhesion Pathway (83067); Focal Adhesion Pathway (478); MAPK Signaling Pathway (83048); MAPK Signaling Pathway (456); Proteoglycans In Cancer Pathway (782000); Proteoglycans In Cancer Pathway (782054); Salmonella Infection Pathway (375172)
ncbi summary :
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]
uniprot summary :
FLNC: 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. Defects in FLNC are the cause of myopathy myofibrillar type 5 (MFM5). A neuromuscular disorder, usually with an adult onset, characterized by focal myofibrillar destruction and pathological cytoplasmic protein aggregations, and clinical features of a limb-girdle myopathy. Defects in FLNC are the cause of myopathy distal type 4 (MPD4). MPD4 is a slowly progressive muscular disorder characterized by distal muscle weakness and atrophy affecting the upper and lower limbs. Onset occurs around the third to fourth decades of life, and patients remain ambulatory even after long disease duration. Muscle biopsy shows non-specific changes with no evidence of rods, necrosis, or inflammation. Belongs to the filamin family. 2 isoforms of the human protein are produced by alternative splicing. Protein type: Cytoskeletal; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 7q32.1. Cellular Component: costamere; cytoplasm; cytoskeleton; cytosol; focal adhesion; plasma membrane; sarcolemma; sarcoplasm; Z disc. Molecular Function: actin filament binding; ankyrin binding; cytoskeletal protein binding; protein binding. Biological Process: muscle fiber development. Disease: Cardiomyopathy, Familial Hypertrophic, 26; Myopathy, Distal, 4; Myopathy, Myofibrillar, 5