COL4-A4; CA44; Collagen Alpha-4(IV)chain; Collagen Of Basement Membrane, Alpha-4 Chain

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Antigen,specific affinity chromatography followed by Protein A affinity chromatography

Supplied as solution form in PBS, pH7.4, containing 0.02% NaN3, 50% glycerol.

0.45 mg/ml (lot specific)

Storage: Avoid repeated freeze/thaw cycles.
Store at 4oC for frequent use.
Aliquot and store at -20oC for 12 months.

Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-COL4a4 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunoprecipitation (IP).

Western blotting: 0.5-2 ug/mL;1:220-90
Immunohistochemistry: 5-20 ug/mL;1:22-90
Immunocytochemistry: 5-20 ug/mL;1:22-90

Optimal working dilutions must be determined by end user.

Western Blot: Sample: Recombinant protein.

DAB staining on fromalin fixed paraffin- embedded kidney tissue)

collagen type IV alpha 4 chain

collagen alpha-4(IV) chain

Collagen alpha-4(IV) chain

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]

COL4A4: 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. Defects in COL4A4 are a cause of Alport syndrome autosomal recessive (APSAR). 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. Defects in COL4A4 are a cause of benign familial hematuria (BFH); also known as thin basement membrane nephropathy. 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 hematuria and thin GBM at that age. Belongs to the type IV collagen family.

Protein type: Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 2q36.3

Cellular Component: basal lamina; collagen type IV; endoplasmic reticulum lumen; extracellular region

Molecular Function: extracellular matrix structural constituent

Biological Process: collagen catabolic process; extracellular matrix organization; glomerular basement membrane development

Disease: Alport Syndrome, Autosomal Recessive

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