Mouse anti-Human Collagen Type IV, 7S Domain Monoclonal Antibody | anti-COL4A3 antibody
Collagen Type IV, 7S Domain (Arresten, ASLN, ATS, CA54, Canstatin, COL4A1, COL4A2, Collagen Alpha 1(IV) Chain, Collagen Alpha 2(IV) Chain, Collagen IV Alpha 1 Polypeptide, Collagen IV Alpha 2 Polypeptide, Collagen of Basement Membrane Alpha 1 Chain, Colla
Other applications have not been tested
Immunohistochemistry (Frozen):1-10ug/ml
Immunohistochemistry (Paraffin): 1-10ug/ml
Optimal dilutions to be determined by the researcher
NCBI and Uniprot Product Information
NCBI Description
Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. 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. [provided by RefSeq, Jun 2010]
Uniprot Description
COL4A3: 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. Autoantibodies against the NC1 domain of alpha 3(IV) are found in Goodpasture syndrome, an autoimmune disease of lung and kidney. Defects in COL4A3 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 COL4A3 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. Defects in COL4A3 are a cause of Alport syndrome autosomal dominant (APSAD). Alport syndrome 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. Belongs to the type IV collagen family. 5 isoforms of the human protein are produced by alternative splicing.
Protein type: Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 2q36-q37
Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region; basement membrane
Molecular Function: metalloendopeptidase inhibitor activity; integrin binding; protein binding; extracellular matrix structural constituent; structural molecule activity
Biological Process: caspase activation; extracellular matrix disassembly; negative regulation of cell proliferation; collagen catabolic process; axon guidance; cell proliferation; extracellular matrix organization and biogenesis; negative regulation of angiogenesis; sensory perception of sound; cell surface receptor linked signal transduction; blood circulation; glomerular basement membrane development; cell adhesion
Disease: Hematuria, Benign Familial; Alport Syndrome, Autosomal Dominant; Alport Syndrome, Autosomal Recessive
Research Articles on COL4A3
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Product Notes
The COL4A3 col4a3 (Catalog #AAA604088) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Collagen Type IV, 7S Domain (Arresten, ASLN, ATS, CA54, Canstatin, COL4A1, COL4A2, Collagen Alpha 1(IV) Chain, Collagen Alpha 2(IV) Chain, Collagen IV Alpha 1 Polypeptide, Collagen IV Alpha 2 Polypeptide, Collagen of Basement Membrane Alpha 1 Chain, Colla reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's Collagen Type IV, 7S Domain can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC) Other applications have not been tested. Western Blot:2-10ug/ml Detects human type IV collagen from human placental tissues giving a band at approximately 92kD. Immunohistochemistry (Frozen):1-10ug/ml Immunohistochemistry (Paraffin): 1-10ug/ml Optimal dilutions to be determined by the researcher. Researchers should empirically determine the suitability of the COL4A3 col4a3 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "Collagen Type IV, 7S Domain, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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