Collagen alpha-4(IV) chain

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

Natural and recombinant Human Collagen alpha-4(IV) chain

For long term storage, please store the entire kit at -20 degree C.

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Small volumes of COL4A4 elisa kit 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.

MBS2891197 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the COL4A4/Collagen alpha-4(IV) Chain, ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing COL4A4. The ELISA analytical biochemical technique of the MBS2891197 kit is based on COL4A4 antibody-COL4A4 antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect COL4A4 antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, COL4A4. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

collagen type IV alpha 4 chain

collagen alpha-4(IV) chain

Collagen alpha-4(IV) chain

CO4A4_HUMAN

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, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 2q35-q37

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

Molecular Function: extracellular matrix structural constituent

Biological Process: axon guidance; extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; glomerular basement membrane development

Disease: Alport Syndrome, Autosomal Recessive

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