Molecular weight (DA)
160kD
Immunogen
The antiserum was produced against synthesized peptide derived from human Collagen IV alpha6. AA range:1201-1250
Specificity
COL4A6 Polyclonal Antibody detects endogenous levels of COL4A6 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/40000. Not yet tested in other applications.
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Background
This gene encodes one of the six subunits of type IV collagen, the major structural component 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, alpha 5 type IV collagen, so that the gene pair shares a common promoter. Deletions in the alpha 5 gene that extend into the alpha 6 gene result in diffuse leiomyomatosis accompanying the X-linked Alport syndrome caused by the deletion in the alpha 5 gene. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Dec 2013],
Function
disease:Deletions covering the N-terminal regions of COL4A6 and COL4A5, which are localized in a head-to-head manner, are the cause of diffuse leiomyomatosis with Alport syndrome (DL-ATS) [MIM:308940]; also known as esophageal and vulval leiomyomatosis with nephropathy or Alport syndrome and diffuse leiomyomatosis (ATS-DL). DL-ATS is the combination of Alport syndrome (AS) and diffuse leiomyomatosis (DL). AS is characterized by progressive glomerulonephritis, often associated with high-tone sensorineural deafness, specific eye abnormalities (lenticonous and macular flecks), and glomerular basement membrane defects. DL is a tumorous process involving smooth muscle cells, mostly of the esophagus, but also of the tracheobronchial tree and the female genital tract.,domain:Alpha chains of type IV collagen have a non-collagenous domain (NC1) at their C-terminus, frequent interruptions of the G
Protein name
Collagen alpha-6(IV) chain
Other name
COL4A6; Collagen alpha-6(IV) chain
Fields
>>PI3K-Akt signaling pathway;>>Focal adhesion;>>ECM-receptor interaction;>>Relaxin signaling pathway;>>AGE-RAGE signaling pathway in diabetic complications;>>Protein digestion and absorption;>>Amoebiasis;>>Human papillomavirus infection;>>Pathways in cancer;>>Small cell lung cancer
Human protein sequence Database
Q14031
Mouse protein sequence database
Rat protein sequence database
Cellular localization
Secreted, extracellular space, extracellular matrix, basement membrane.
Tissue expression
Eye,Kidney,Prostate,
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