catalog number :
MBS316304
products full name :
Rabbit A' Chicken Collagen III
products short name :
Collagen Type III
products name syn :
Rabbit A'Chicken Collagen III; Rabbit Antibody to Chicken Collagen Type III
other names :
collagen, type III, alpha 1 (Ehlers-Danlos syndrome type IV, autosomal dominant), isoform CRA_b; Collagen alpha-1(III) chain; collagen alpha-1(III) chain; collagen alpha-1(III) chain; collagen, fetal; OTTHUMP00000163485; alpha1 (III) collagen; Ehlers-Danlos syndrome type IV, autosomal dominant; collagen, type III, alpha 1
other gene names :
COL3A1; COL3A1; EDS4A; FLJ34534
uniprot entry name :
CO3A1_HUMAN
specificity :
Collagen Type III. Crossreactivity: (RIA%). Chicken collagen type III - 100%. Chicken collagen type I - <5.0%. Human, Mouse, Rat Collagens, Type III - <0.1%.
purity :
Ion exchange chromatography (DEAE-Trisacryl)
form :
Purified, Lyophilized. Reconstitute with 0.5 ml deionized water.
concentration :
Not determined. Titer: 1:1000 (RIA)
storage stability :
Lyophilized: Store (up to 24 months) at 2 to 8 degree C. Long term store at -20 degree C. Reconstituted: Aliquot and store at -20 degree C. Avoid multiple freeze/thaw cycles.
app notes :
IFA, (SP)RIA and ELISA. For immunostaining of extra or intracellular components in light microscopy. For the quantitation of Collagen Type III in biological fluids or in culture supernatants. In indirect IFA, use diluted at >1:40 on frozen chicken tissue
other info1 :
Immunogen: Collagen type III extracted and purified from chicken skin
other info2 :
Buffer: Not applicable. Preservative: No. Lyophilized: Yes. Important Note: Centrifuge before opening to ensure complete recovery of vial contents.
products categories :
Polyclonal Antibodies to Collagens and Extracellular Matrix Proteins
ncbi mol weight :
138,564 Da
ncbi pathways :
Amoebiasis Pathway 167324!!Amoebiasis Pathway 167191!!Axon Guidance Pathway 105688!!ECM-receptor Interaction Pathway 83068!!ECM-receptor Interaction Pathway 479!!Endothelins Pathway 137958!!Focal Adhesion Pathway 198795!!Focal Adhesion Pathway 83067!!Focal Adhesion Pathway 478!!Inflammatory Response Pathway 198766
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]
uniprot summary :
Function: Collagen type III occurs in most soft connective tissues along with type I collagen. Subunit structure: 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. Subcellular location: Secreted extracellular space extracellular matrix. Post-translational modification: Proline residues at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.O-linked glycan consists of a Glc-Gal disaccharide bound to the oxygen atom of a post-translationally added hydroxyl group. Involvement in 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. Ref.44Defects 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. Ref.30 Ref.31 Ref.32 Ref.33 Ref.34 Ref.35 Ref.37 Ref.38 Ref.39 Ref.40 Ref.41 Ref.43 Ref.45 Ref.46 Ref.47 Ref.48 Ref.49 Ref.50 Ref.51 Ref.52 Ref.53 Ref.55 Ref.56 Ref.57 Ref.58 Ref.60Defects 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. Ref.27 Ref.29 Ref.36. Sequence similarities: Belongs to the fibrillar collagen family.Contains 1 fibrillar collagen NC1 domain.Contains 1 VWFC domain.