Mouse Collagen III/COL3A1 Monoclonal Antibody | anti-COL3A1 antibody
Anti-Collagen III/COL3A1 Antibody (Monoclonal, 9H9)
IHC-P: 0.5-1ug/ml
Background: COL3A1, also called EDS4A or Collagen alpha-1(III), is a protein that in humans is encoded by the COL3A1 gene. It is mapped to 2q32.2. COL3A1 chain is a fibrillar-forming collagen comprising 3 alpha-1(III) chains and is expressed in early embryos and throughout embryogenesis. In adult, COL3A1 is a major component of the extracellular matrix in a variety of internal organs and skin. COL3A1 is also a fibrous scleroprotein in bone, cartilage, dentin, tendon, bone marrow stroma and other connective tissue. It is involved in regulation of cortical development, and it is the major ligand of GPR56 in the developing brain. COL3A1 binding to GPR56 can inhibit neuronal migration and activate the RhoA pathway by coupling GPR56 to GNA13 and possibly GNA12.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes the pro-alpha1 chains of type III collagen, a fibrillar collagen that is found in extensible connective tissues such as skin, lung, uterus, intestine and the vascular system, frequently in association with type I collagen. Mutations in this gene are associated with Ehlers-Danlos syndrome types IV, and with aortic and arterial aneurysms. Two transcripts, resulting from the use of alternate polyadenylation signals, have been identified for this gene. [provided by R. Dalgleish, Feb 2008]
Uniprot Description
CO3A1: Collagen type III occurs in most soft connective tissues along with type I collagen. Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3); also known as benign hypermobility syndrome. EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS3 is a form of Ehlers-Danlos syndrome characterized by marked joint hyperextensibility without skeletal deformity. Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4). EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDS4 is the most severe form of the disease. It is characterized by the joint and dermal manifestations as in other forms of the syndrome, characteristic facial features (acrogeria) in most patients, and by proneness to spontaneous rupture of bowel and large arteries. The vascular complications may affect all anatomical areas. Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA). AAA is a common multifactorial disorder characterized by permanent dilation of the abdominal aorta, usually due to degenerative changes in the aortic wall. Histologically, AAA is characterized by signs of chronic inflammation, destructive remodeling of the extracellular matrix, and depletion of vascular smooth muscle cells. Belongs to the fibrillar collagen family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: Extracellular matrix; Secreted; Secreted, signal peptide; Motility/polarity/chemotaxis; Cell adhesion
Chromosomal Location of Human Ortholog: 2q31
Cellular Component: extracellular matrix; extracellular space; endoplasmic reticulum lumen; extracellular region; collagen type III
Molecular Function: integrin binding; protein binding; extracellular matrix structural constituent; metal ion binding; platelet-derived growth factor binding; SMAD binding
Biological Process: skin development; integrin-mediated signaling pathway; receptor-mediated endocytosis; axon guidance; platelet activation; extracellular matrix organization and biogenesis; collagen fibril organization; wound healing; heart development; cell-matrix adhesion; negative regulation of immune response; positive regulation of Rho protein signal transduction; extracellular matrix disassembly; collagen catabolic process; response to radiation; gut development; response to mechanical stimulus; response to cytokine stimulus; transforming growth factor beta receptor signaling pathway; fibril organization and biogenesis; cerebral cortex development; peptide cross-linking; skeletal development; aging
Disease: Ehlers-danlos Syndrome, Type Iv, Autosomal Dominant; Ehlers-danlos Syndrome, Type Iii
Research Articles on COL3A1
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Product Notes
The COL3A1 col3a1 (Catalog #AAA1752797) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The Anti-Collagen III/COL3A1 Antibody (Monoclonal, 9H9) reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's Collagen III/COL3A1 can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC) Paraffin. WB: 0.1-0.5ug/ml IHC-P: 0.5-1ug/ml. Researchers should empirically determine the suitability of the COL3A1 col3a1 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 III/COL3A1, 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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