catalog number :
MBS634533
products full name :
Collagen Type III, Human
products short name :
[Collagen Type III]
other names :
[collagen type III alpha 1]
specificity :
Reacts with anti-Collagen Type III. Reaction with anti-Collagen I, II, IV, V or VI is negligible (typically less than 1% cross reactivity was detected by ELISA).
purity :
Purified by immunoaffinity chromatography. It is supplied free from other collagens, human serum proteins, and non-collagen extracellular matrix proteins
form :
Supplied as a liquid in 0.5M acetic acid, 0.01% sodium azide. No stabilizing proteins added.
storage stability :
May be stored at 4°C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and freeze at -20°C or colder. Aliquots are stable for at least 6 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
tested application :
ELISA (EL/EIA), Western Blot (WB), Immunoprecipitation (IP) - Other applications not tested.
app notes :
ELISA: 1:10,000. Western Blot: 1:1000. Immunochemistry: 1:100-1:500. Optimal diluitons to be determined by the researcher.
other info1 :
Grade: Affinity Purified
products categories :
Molecular Biology; MB-Collagen
ncbi acc num :
AGL34959.1
ncbi summary :
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 summary :
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: Cell adhesion; Secreted; Secreted, signal peptide; Motility/polarity/chemotaxis; Extracellular matrix. 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; platelet-derived growth factor binding; extracellular matrix structural constituent; metal ion binding; SMAD binding. Biological Process: integrin-mediated signaling pathway; skin development; 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; collagen catabolic process; extracellular matrix disassembly; response to radiation; gut development; response to mechanical stimulus; response to cytokine stimulus; transforming growth factor beta receptor signaling pathway; fibril organization and biogenesis; peptide cross-linking; cerebral cortex development; skeletal development; aging. Disease: Ehlers-danlos Syndrome, Type Iv, Autosomal Dominant; Ehlers-danlos Syndrome, Type Iii