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ITM3123

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ITM3123
  • Catalog: ITM3123
  • Gene/Protein: COL3A1
  • Product Description: Immunotag™ Collagen III Monoclonal Antibody(Q76)
405.0000
Price in reward points: 405

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Immunotag™ Collagen III Monoclonal Antibody(Q76)
Antibody Specification
Datasheet
Target Protein Collagen III (Q76)
Clonality Monoclonal
Storage/Stability -20°C/1 year
Application WB,IHC-p,IF,
Recommended Dilution WB: 1:1000 IF 1:200 IHC 1:50-300
Concentration 1 mg/ml
Reactive Species Human,Mouse,Rat
Host Species Mouse
Immunogen Synthetic Peptide of Collagen III
Specificity The antibody detects endogenous Collagen III protein.
Purification The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen
Form PBS, pH 7.4, containing 0.02% sodium azide as Preservative and 50% Glycerol.
Gene Name COL3A1
Accession No. P02461 P08121 P13941
Alternate Names COL3A1; Collagen alpha-1(III) chain
Description collagen type III alpha 1 chain(COL3A1) Homo sapiens 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],
Cell Pathway/ Category Focal adhesion,ECM-receptor interaction,
Protein Expression Colon carcinoma,Liver,Placenta,Skin fibroblast,
Subcellular Localization extracellular region,collagen trimer,collagen type III trimer,extracellular space,endoplasmic reticulum lumen,extracellular matrix,
Protein Function disease:Defects in COL3A1 are a cause of Ehlers-Danlos syndrome type 3 (EDS3) [MIM:130020]; 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.,disease:Defects in COL3A1 are a cause of susceptibility to aortic aneurysm abdominal (AAA) [MIM:100070]. 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.,disease:Defects in COL3A1 are the cause of Ehlers-Danlos syndrome type 4 (EDS4) [MIM:130050]. 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.,function:Collagen type III occurs in most soft connective tissues along with type I collagen.,online information:Collagen type III alpha-1 chain mutations,online information:Type-III collagen entry,PTM:O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group.,PTM:Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.,similarity:Belongs to the fibrillar collagen family.,similarity:Contains 1 VWFC domain.,subunit:Trimers of identical alpha 1(III) chains. The chains are linked to each other by interchain disulfide bonds. Trimers are also cross-linked via hydroxylysines.,
Usage For Research Use Only! Not for diagnostic or therapeutic procedures.
Material Safety Data Sheet
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