Anti-FLNC 항체 (CAB13018)

Antibody Name:	Anti-FLNC Antibody
Antibody SKU:	CAB13018
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 2160-2340 of human FLNC (NP_001449.3).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse
Positive Samples:	A-549, LO2, NIH/3T3

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 2160-2340 of human FLNC (NP_001449.3).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	IPGN WFQM VSAQ ERLT RTFT RSSH TYTR TERT EISK TRGG ETKR EVRV EEST QVGG DPFP AVFG DFLG RERL GSFG SITR QQEG EASS QDMT AQVT SPSG KVEA AEIV EGED SAYS VRFV PQEM GPHT VAVK YRGQ HVPG SPFQ FTVG PLGE GGAH KVRA GGTG LERG VAGV PAEF SIWT R
Gene ID:	2318
Uniprot:	Q14315
Cellular Location:	Cytoplasm, Membrane, Peripheral membrane protein, Z line, cytoskeleton, myofibril, sarcomere
Calculated MW:	287kDa/291kDa
Observed MW:	302kDa

Synonyms:

FLNC, ABP-280, ABP280A, ABPA, ABPL, CMH26, FLN2, MFM5, MPD4, RCM5, filamin-C

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.

UniProt Protein Function:	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.
UniProt Protein Details:	Protein type:Motility/polarity/chemotaxis; Cytoskeletal Chromosomal Location of Human Ortholog: 7q32-q35 Cellular Component: costamere; focal adhesion; cytoskeleton; cytoplasm; plasma membrane; sarcoplasm; Z disc; cytosol; sarcolemma Molecular Function:actin filament binding; protein binding; cytoskeletal protein binding; ankyrin binding Biological Process: muscle fiber development Disease: Myopathy, Myofibrillar, 5; Myopathy, Distal, 4
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 Code:	Q14315
NCBI GenInfo Identifier:	254763419
NCBI Gene ID:	2318
NCBI Accession:	Q14315.3
UniProt Secondary Accession:	Q14315,O95303, Q07985, Q9NS12, Q9NYE5, Q9UMR8, Q9Y503 B2ZZ88,
UniProt Related Accession:	Q14315
Molecular Weight:	291,022 Da
NCBI Full Name:	Filamin-C
NCBI Synonym Full Names:	filamin C, gamma
NCBI Official Symbol:	FLNC
NCBI Official Synonym Symbols:	ABPA; ABPL; FLN2; MFM5; MPD4; ABP-280; ABP280A
NCBI Protein Information:	filamin-C; filamin-2; ABP-280-like protein; ABP-L, gamma filamin; actin binding protein 280
UniProt Protein Name:	Filamin-C
UniProt Synonym Protein Names:	ABP-280-like protein; ABP-L; Actin-binding-like protein; Filamin-2; Gamma-filamin
Protein Family:	Filamin
UniProt Gene Name:	FLNC
UniProt Entry Name:	FLNC_HUMAN

	Western blot analysis of extracts of various cell lines, using FLNC antibody (CAB13018) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 1s.
	Immunofluorescence analysis of HeLa cells using FLNC antibody (CAB13018) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.

ELISA Kits

Human Filamin-C ELISA Kit

Human FLNC (Filamin C, Gamma) CLIA Kit (HUES01118)

Human Filamin-C (FLNC) ELISA Kit

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)