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Collagen IV Recombinant Rabbit mAb (bsm-56208R)  
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產(chǎn)品編號 bsm-56208R
英文名稱 Collagen IV Recombinant Rabbit mAb
中文名稱 IV型膠原蛋白重組兔單抗
別    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; Collagen Ⅳ; Collagen Type Ⅳ.  
抗體來源 Rabbit
克隆類型 Recombinant
克 隆 號 90000
交叉反應(yīng) Human
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 161 kDa
檢測分子量
細(xì)胞定位 細(xì)胞膜 細(xì)胞外基質(zhì) 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human Collagen IV 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 PBS (pH7.2) with 40% Glycerol, 0.05% BSA and 0.02% Proclin300.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 This gene encodes the major type IV alpha collagen chain of basement membranes. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. [provided by RefSeq, Jul 2008]

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.

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.

SWISS:
P02462

Gene ID:
1282

Database links:

Entrez Gene: 1282 Human

Omim: 120070 Human

SwissProt: P02462 Human

Unigene: 17441 Human



IV型膠原為基底膜的主要成分,主要用于各種良惡性組織(乳腺癌、胃腸道癌等)中基底膜分布情況的研究.IV型膠原是構(gòu)成基底膜的主要成分。該抗體可特異性識別人的IV型膠原,與人類皮膚、腎、肌肉、脾、淋巴結(jié)、胎盤和肺的基底膜呈陽性反應(yīng),主要用于各種組織癌癥中基底膜情況的研究。
產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (human liver carcinoma); Antigen retrieval by boiling in sodium EDTA buffer (Ph9.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (Collagen IV) Monoclonal Antibody, Unconjugated (bsm-56208R) at 1:500 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
Paraformaldehyde-fixed, paraffin embedded (human lung carcinoma); Antigen retrieval by boiling in sodium EDTA buffer (Ph9.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Incubation with (Collagen IV) Monoclonal Antibody, Unconjugated (bsm-56208R) at 1:500 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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