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Mouse Anti-Collagen IV/FITC Conjugated antibody
background:
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 SLCterminal NC1 domain, inhibits angiogenesis and tumor formation. The SLCterminal 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 SLCterminal 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.
Database links:
Entrez Gene: 1282 Human
Omim: 24070 Human
SwissProt: P02462 Human
Unigene: 17441 Human
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
IV型胶原为基底膜的主要成分，主要用于各种良恶性组织(乳腺癌、胃肠道癌等)中基底膜分布情况的研究.IV型胶原是构成基底膜的主要成分。该抗体可特异性识别人的IV型胶原，与人类皮肤、肾、肌肉、脾、淋巴结、胎盘和肺的基底膜呈阳性反应，主要用于各种组织癌症中基底膜情况的研究。
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