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Cell Signal Transduction Research Antibodies
COL4A4 Polyclonal AntibodyAntibody
Polyclonal, Rabbit
$225/ 100ul
QuantityTotal: $225
Ship worldwide. Contact order@enogene.com for ordering and bulk pricing or contact your local distributor.
Product Information
Molecular Weight163999Da
Protein NameCollagen alpha-4(IV) chain
Human Gene Id1286
Human Swiss Prot NoP53420
Mouse Swiss Prot NoQ9QZR9
SpecificityCOL4A4 Polyclonal Antibody detects endogenous levels of COL4A4 protein.
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Concentration1 mg/ml
Storage Stability-20°C/1 year
Other NameCOL4A4; Collagen alpha-4(IV) chain
Functiondisease:Defects in COL4A4 are a cause of Alport syndrome autosomal recessive (APSAR) [MIM:203780]. APSAR is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness.,disease:Defects in COL4A4 are a cause of benign familial hematuria (BFH) [MIM:141200]; also known as thin basement membrane disease. BFH is characterized by persistent hematuria, an electron microscopically detectable thin glomerular basement membrane (GBM) and an autosomal dominant mode of inheritance. Renal function remains normal. In children, differentiation between BFH and AS can be difficult, because both disorders are manifested by persistent hematuri
Subcellular Locationextracellular region,proteinaceous extracellular matrix,collagen trimer,collagen type IV trimer,basal lamina,endoplasmic reticulum lumen,
ExpressionEye,Kidney,
PurificationThe antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
ImmunogenThe antiserum was produced against synthesized peptide derived from human Collagen IV alpha4. AA range:541-590
Gene NameCOL4A4
DilutionImmunohistochemistry: 1/100 - 1/300. Immunofluorescence: 1/200 - 1/1000. ELISA: 1/5000. Not yet tested in other applications.
Backgroundcollagen type IV alpha 4 chain(COL4A4) Homo sapiens This gene encodes one of the six subunits of type IV collagen, the major structural component of basement membranes. This particular collagen IV subunit, however, is only found in a subset 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. Mutations in this gene are associated with type II autosomal recessive Alport syndrome (hereditary glomerulonephropathy) and with familial benign hematuria (thin basement membrane disease). Two transcripts, differing only in their transcription start sites, have been identified for this gene and, as is common for collagen genes, multiple polyadenylation sites are found in the 3' UTR. [provided by RefSeq, Jul 2008],
Conjugated Antibody
This product is also available conjugated. Format: E20-71027-Conjugate. Price: $485 / Size: 100μl.
E20-71027-FITC$485 / 100μlE20-71027-Biotin$485 / 100μlE20-71027-HRP$485 / 100μlE20-71027-AP$485 / 100μlE20-71027-AF350$485 / 100μlE20-71027-AF405M$485 / 100μlE20-71027-AF488$485 / 100μlE20-71027-AF514$485 / 100μlE20-71027-AF532$485 / 100μlE20-71027-AF555$485 / 100μlE20-71027-AF568$485 / 100μlE20-71027-AF405S$485 / 100μlE20-71027-AF405L$485 / 100μlE20-71027-AF546$485 / 100μlE20-71027-AF594$485 / 100μlE20-71027-AF610$485 / 100μlE20-71027-AF635$485 / 100μlE20-71027-AF647$485 / 100μlE20-71027-AF680$485 / 100μlE20-71027-AF700$485 / 100μlE20-71027-AF750$485 / 100μlE20-71027-AF790$485 / 100μlE20-71027-APC$485 / 100μlE20-71027-PE$485 / 100μlE20-71027-Cy3$485 / 100μlE20-71027-Cy5$485 / 100μlE20-71027-Cy5.5$485 / 100μlE20-71027-Cy7$485 / 100μlE20-71027-PE-Cy5$485 / 100μl
Contact order@enogene.com to order conjugated formats. Magnetic Beads (1-30μm) conjugation is also available.
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