Molecular weight (DA)
160kD
Immunogen
The antiserum was produced against synthesized peptide derived from human Collagen IV alpha5. AA range:21-70
Specificity
COL4A5 Polyclonal Antibody detects endogenous levels of COL4A5 protein.
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000 Immunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/10000. 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. Mutations in this gene are associated with X-linked Alport syndrome, also known as hereditary nephritis. 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. Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Aug 2010],
Function
disease:Defects in COL4A5 are the cause of Alport syndrome X-linked (APSX) [MIM:301050]. APSX is characterized by progressive glomerulonephritis, renal failure, sensorineural deafness, specific eye abnormalities (lenticonous and macular flecks), and glomerular basement membrane defects. The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness.,disease:Deletions covering the N-terminal regions of COL4A5 and COL4A6, 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 AS and diffuse leiomyomatosis (DL). DL is a tumorous process involving smooth muscle cells, mostly of the esophagus,
Protein name
Collagen alpha-5(IV) chain
Other name
COL4A5; Collagen alpha-5(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
P29400
Mouse protein sequence database
Rat protein sequence database
Cellular localization
Secreted, extracellular space, extracellular matrix, basement membrane.
Tissue expression
Isoform 2 is found in kidney.
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