产品名称
Integrin β4 Rabbit Polyclonal Antibody
别名
ITGB4; Integrin beta-4; GP150; CD antigen CD104
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=3691
Human Swissprot No.
P16144
Human Swissprot Link
http://www.uniprot.org/uniprotkb/P16144/entry
Mouse Swissprot No.
A2A863
Mouse Swissprot Link
http://www.uniprot.org/uniprot/A2A863
Rat Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=25724
Rat Swissprot Link
http://www.uniprot.org/uniprot/Q64632
免疫原
The antiserum was produced against synthesized peptide derived from human Integrin beta4. AA range:1481-1530
特异性
Integrin β4 Polyclonal Antibody detects endogenous levels of Integrin β4 protein.
稀释度
WB 1:500 - 1:2000. IHC 1:100 - 1:300. ELISA: 1:20000.. IF 1:50-200
宿主
Polyclonal, Rabbit,IgG
背景介绍
Integrins are heterodimers comprised of alpha and beta subunits, that are noncovalently associated transmembrane glycoprotein receptors. Different combinations of alpha and beta polypeptides form complexes that vary in their ligand-binding specificities. Integrins mediate cell-matrix or cell-cell adhesion, and transduced signals that regulate gene expression and cell growth. This gene encodes the integrin beta 4 subunit, a receptor for the laminins. This subunit tends to associate with alpha 6 subunit and is likely to play a pivotal role in the biology of invasive carcinoma. Mutations in this gene are associated with epidermolysis bullosa with pyloric atresia. Multiple alternatively spliced transcript variants encoding distinct isoforms have been found for this gene. [provided by RefSeq, Jul 2008],
组织表达
Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoform beta-4D is also expressed in colon and placenta. Isoform beta-4E is also expressed in epidermis, lung, duodenum, heart, spleen and stomach.
细胞定位
Cell membrane; Single-pass type I membrane protein. Cell membrane; Lipid-anchor. Cell junction, hemidesmosome. Colocalizes with DST at the leading edge of migrating keratinocytes.
信号通路
Focal adhesion;ECM-receptor interaction;Regulates Actin and Cytoskeleton;Hypertrophic cardiomyopathy (HCM);Arrhythmogenic right ventricular cardiomyopathy (ARVC);Dilated cardiomyopathy;
功能
disease:Defects in ITGB4 are a cause of epidermolysis bullosa letalis with pyloric atresia (EB-PA) [MIM:226730]; also known as junctional epidermolysis bullosa with pyloric atresia (PA-JEB) or aplasia cutis congenita with gastrointestinal atresia. EB-PA is an autosomal recessive, frequently lethal, epidermolysis bullosa with variable involvement of skin, nails, mucosa, and with variable effects on the digestive system. It is characterized by mucocutaneous fragility, aplasia cutis congenita, and gastrointestinal atresia, which most commonly affects the pylorus. Pyloric atresia is a primary manifestation rather than a scarring process secondary to epidermolysis bullosa.,disease:Defects in ITGB4 are a cause of generalized atrophic benign epidermolysis bullosa (GABEB) [MIM:226650]. GABEB is a non-lethal, adult form of junctional epidermolysis bullosa characterized by life-long blistering of the skin, associated with hair and tooth abnormalities.,domain:The fibronectin type-III-like domains bind BPAG1 and plectin and probably also recruit BP230.,function:Integrin alpha-6/beta-4 is a receptor for laminin. It plays a critical structural role in the hemidesmosome of epithelial cells.,similarity:Belongs to the integrin beta chain family.,similarity:Contains 1 Calx-beta domain.,similarity:Contains 1 VWFA domain.,similarity:Contains 4 fibronectin type-III domains.,subunit:Heterodimer of an alpha and a beta subunit. Beta-4 associates with alpha-6.,tissue specificity:Integrin alpha-6/beta-4 is predominantly expressed by epithelia. Isoform beta-4D is also expressed in colon and placenta. Isoform beta-4E is also expressed in epidermis, lung, duodenum, heart, spleen and stomach.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.