Product Description

Filamin 1 Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES2345-50, ES2345-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: filamin A(FLNA) Homo sapiens The protein encoded by this gene is an actin-binding protein that crosslinks actin filaments and links actin filaments to membrane glycoproteins. The encoded protein is involved in remodeling the cytoskeleton to effect changes in cell shape and migration. This protein interacts with integrins, transmembrane receptor complexes, and second messengers. Defects in this gene are a cause of several syndromes, including periventricular nodular heterotopias (PVNH1, PVNH4), otopalatodigital syndromes (OPD1, OPD2), frontometaphyseal dysplasia (FMD), Melnick-Needles syndrome (MNS), and X-linked congenital idiopathic intestinal pseudoobstruction (CIIPX). Two transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2009],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IHC, IF, ELISA

Alternate Names: FLNA, FLN, FLN1, Filamin-A, FLN-A, Actin-binding protein 280, ABP-280, Alpha-filamin, Endothelial actin-binding protein, Filamin-1, Non-muscle filamin

Clonality: Polyclonal

Immunogen: The antiserum was produced against synthesized peptide derived from human Filamin A. AA range:2121-2170

Isotype: IgG

Target: Filamin 1

Specificity: Filamin 1 Polyclonal Antibody detects endogenous levels of Filamin 1 protein.

Dilution: Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/10000. Not yet tested in other applications.

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Expression: Ubiquitous,

Observed Band (KD): 280kD

Fields: MAPK signaling pathway, Focal adhesion, Salmonella infection, Proteoglycans in cancer

Function: Disease: Defects in FLNA are associated with cerebrofrontofacial syndrome [MIM: 608578]. This syndrome consists of a phenotype of male PVNH, with relatively normal development, no epilepsy or other neurological abnormality, severe constipation, and facial dysmorphism and without a discernible skeletal phenotype
Disease: Defects in FLNA are the cause of frontometaphyseal dysplasia (FMD) [MIM: 305620]. FMD is a congenital bone disease characterized by supraorbital hyperostosis, deafness and digital anomalies
Disease: Defects in FLNA are the cause of Melnick-Needles syndrome (MNS) [MIM: 309350]. MNS is a severe congenital bone disorder characterized by typical facies (exophthalmos, full cheeks, micrognathia and malalignment of teeth), flaring of the metaphyses of long bones, s-like curvature of bones of legs, irregular constrictions in the ribs, and sclerosis of base of skull
Disease: Defects i

Subcellular Location: Cytoplasm, cell cortex Cytoplasm, cytoskeleton Perikaryon Cell projection, growth cone Colocalizes with CPMR1 in the central region of DRG neuron growth cone (By similarity) Following SEMA3A stimulation of DRG neurons, colocalizes with F-actin (By similarity)

Storage and Stability: -20°C/1 year

Gene Name: FLNA

Protein Name: Filamin-A

Mouse Gene ID: 192176

Mouse SwissProt Number: Q8BTM8

Research Use Only