Collagen IV,IV型胶原蛋白/4型胶原蛋白/胶原蛋白4抗体

产品编号HZ-4595R
英文名称Collagen IV
中文名称IV型胶原蛋白/4型胶原蛋白/胶原蛋白4抗体
别 名Arresten; Canstatin; COL4A1; HANAC; ICH; POREN1; Collagen Alpha 1(IV) Chain; Collagen IV Alpha 1 Polypeptide; Collagen Of Basement Membrane Alpha 1 Chain; Collagen Of Basement Membrane Alpha 2 Chain; Collagen Type IV Alpha 1; DKFZp686I14213; FLJ22259; collagen alpha-1(IV) chain preproprotein; collagen alpha-1(IV) chain preproprotein; Col4a1 protein; collagen of basement membrane, alpha-1 chain; collagen IV, alpha-1 polypeptide; collagen alpha-1(IV) chain; COL4A1 NC1 domain; CO4A1_HUMAN.
说 明 书0.1ml 0.2ml
研究领域肿瘤 免疫学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
Collagen IV,IV型胶原蛋白/4型胶原蛋白/胶原蛋白4抗体产品应用WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-300 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量165kDa
细胞定位细胞膜 细胞外基质 分泌型蛋白
性 状Lyophilized or Liquid
浓 度1mg/1ml
免 疫 原KLH conjugated synthetic peptide derived from human Collagen alpha-1(IV) chain
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Collagen IV,IV型胶原蛋白/4型胶原蛋白/胶原蛋白4抗体PubMedPubMed
产品介绍background:
Collagen IV is a major constituent of the basement membranes along with laminins, proteoglycans and enactins. It is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. It can form insoluble fibers with high tensile strength. Collagen IV is useful in detecting the loss of parts of basement membranes in carcinomas.

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.
Arresten, comprising the C-terminal NC1 domain, inhibits angiogenesis and tumor formation. The C-terminal half is found to possess the anti-angiogenic activity. Specifically inhibits endothelial cell proliferation, migration and tube formation. Inhibits expression of hypoxia-inducible factor 1alpha and ERK1/2 and p38 MAPK activation. Ligand for alpha1/beta1 integrin.

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.

Subcellular Location:
Secreted, extracellular space, extracellular matrix, basement membrane.

Tissue Specificity:
Highly expressed in placenta.

Post-translational modifications:
Lysines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in all cases and bind carbohydrates.
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
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.
The trimeric structure of the NC1 domains is stabilized by covalent bonds between Lys and Met residues.
Proteolytic processing produces the C-terminal NC1 peptide, arresten.

Collagen IV,IV型胶原蛋白/4型胶原蛋白/胶原蛋白4抗体DISEASE:
Defects in COL4A1 are a cause of brain small vessel disease with hemorrhage (BSVDH) [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.
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.
Defects in COL4A1 are a cause of familial porencephaly (POREN1) [MIM:175780]. 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. Type 2, or schizencephalic porencephaly, is usually symmetric and represents a primary defect or arrest in the development of the cerebral ventricles.

Similarity:
Contains 1 FAD-binding FR-type domain.
Contains 1 ferric oxidoreductase domain.

Gene ID:
1282

Database links:
Entrez Gene: 282191 Cow
Entrez Gene: 317711 Cow
Entrez Gene: 407107 Cow
Entrez Gene: 508632 Cow
Entrez Gene: 511602 Cow
Entrez Gene: 1282 Human
Entrez Gene: 1284 Human
Entrez Gene: 1285 Human
Entrez Gene: 1286 Human
Entrez Gene: 1287 Human
Entrez Gene: 12826 Mouse
Entrez Gene: 12827 Mouse
Entrez Gene: 12828 Mouse
Entrez Gene: 12829 Mouse
Entrez Gene: 12830 Mouse
Entrez Gene: 397502 Pig
Entrez Gene: 290905 Rat
Entrez Gene: 301562 Rat
Entrez Gene: 306628 Rat
Entrez Gene: 363265 Rat
Entrez Gene: 363457 Rat
Omim: 120070 Human
Omim: 120090 Human
Omim: 120130 Human
Omim: 120131 Human
Omim: 303630 Human
SwissProt: P02462 Human
SwissProt: P08572 Human
SwissProt: P29400 Human
SwissProt: P53420 Human
SwissProt: Q01955 Human
SwissProt: P02463 Mouse
SwissProt: P08122 Mouse
SwissProt: Q9QZR9 Mouse
SwissProt: Q9QZS0 Mouse
Unigene: 17441 Human
Unigene: 369089 Human
Unigene: 570065 Human
Unigene: 591645 Human
Unigene: 738 Mouse
Unigene: 121139 Rat
Unigene: 22075 Rat
Unigene: 53801

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.