Collagen IV α1 (Cleaved-Gly173) rabbit pAb

产品详情

产品名称

Collagen IV α1 (Cleaved-Gly173) rabbit pAb

产品货号

YM-A03984

规格

50μL;100μL

种属反应

Human,Mouse

实验应用

WB,ELISA

分子量

18kD,185kD

宿主

Rabbit

同种型

IgG

修饰

Unmodified

推荐稀释比

WB 1:1000-2000;ELISA 1:5000-20000

组成

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

纯化工艺

The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

储存

-15°C to -25°C/1 year(Do not lower than -25°C)

浓度

1 mg/ml

克降性

Polyclonal

克隆号

免疫原

Synthesized peptide derived from human Collagen IV α1 (Cleaved-Gly173)

特异性

This antibody detects endogenous levels of Human,Mouse Collagen IV α1 (Cleaved-Gly173, protein was cleaved amino acid sequence between 172-173 )

基因名称

COL4A1

蛋白名称

Collagen IV α1 (Cleaved-Gly173)

别名

Collagen alpha-1;IV;chain [Cleaved into: Arresten]

基因ID-1

1282

SwissProt

P02462

背景

disease:Defects in COL4A1 are a cause of brain small vessel disease with hemorrhage [MIM:607595]. Brain small vessel diseases underlie 20 to 30 percent of ischemic strokes and a larger proportion of intracerebral hemorrhages. Inheritance is autosomal dominant.,disease:Defects in COL4A1 are a cause of porencephaly type 1 [MIM:175780]; also known as encephaloclastic porencephaly. Porencephaly is a term used for any cavitation or cerebrospinal fluid-filled cyst in the brain. Porencephaly type 1 is usually unilateral and results from focal destructive lesions such as fetal vascular occlusion or birth trauma. Inheritance is autosomal dominant.,disease:Defects in COL4A1 are the cause of hereditary angiopathy with nephropathy, aneurysms, and muscle cramps (HANAC) [MIM:611773]. The clinical renal manifestations include hematuria and bilateral large cysts. Histologic analysis revealed complex basement membrane defects in kidney and skin. The systemic angiopathy appears to affect both small vessels and large arteries.,domain:Alpha chains of type IV collagen have a non-collagenous domain (NC1) at their C-terminus, frequent interruptions of the G-X-Y repeats in the long central triple-helical domain (which may cause flexibility in the triple helix), and a short N-terminal triple-helical 7S domain.,function:Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Potently inhibits endothelial cell proliferation and angiogenesis. Inhibits angiogenesis potentially via mechanisms involving cell surface proteoglycans and the alpha and beta integrins of endothelial cells.,PTM:Lysines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in all cases and bind carbohydrates.,PTM:Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,PTM:The trimeric structure of the NC1 domains may be stabilized by covalent bonds between Lys and Met residues.,PTM:Type IV collagens contain numerous cysteine residues which are involved in inter- and intramolecular disulfide bonding. 12 of these, located in the NC1 domain, are conserved in all known type IV collagens.,similarity:Belongs to the type IV collagen family.,similarity:Contains 1 collagen IV NC1 (C-terminal non-collagenous) domain.,subunit:There are six type IV collagen isoforms, alpha 1(IV)-alpha 6(IV), each of which can form a triple helix structure with 2 other chains to generate type IV collagen network.,tissue specificity:Highly expressed in placenta.,

细胞定位

Secreted, extracellular space, extracellular matrix, basement membrane .