Rabbit anti-Human Collagen Type III Alpha 1 (COL3a1) Polyclonal Antibody | anti-COL3a1 antibody

APC-Linked Polyclonal Antibody to Collagen Type III Alpha 1 (COL3a1)

Cat. Num. AAA2070506

• Gene Names: COL3A1; EDS4A
• Reactivity: Human
• Applications: Western Blot, Immunocytochemistry, Immunohistochemistry, ELISA
• Synonyms: Collagen Type III Alpha 1 (COL3a1); Polyclonal Antibody; APC-Linked Polyclonal Antibody to Collagen Type III Alpha 1 (COL3a1); COL3-A1; COL3A1; EDS4A; Collagen Type III Alpha 1; Ehlers-Danlos Syndrome Type IV Autosomal Dominant; Collagen Alpha-1 (III)chain; anti-COL3a1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Concentration: >=100ug/ml (varies by lot)

• Applicable Applications for anti-COL3a1 antibody: Western Blot (WB), Immunocytochemistry (ICC), Immunohistochemistry (IHC) Formalin/Paraffin, ELISA (ELISA)
• Immunogen: COL3a1 (Gly1059~Leu1466)
• Conjugation: APC
• Cross Reactivity: Human, Pig
• Unconjugated Antibody: The unconjugated antibody version of this item is also available as catalog #MBS2004036

Knockout Validation

(Knockout Validation: Lane 1: Wild-type BXPC3 cell lysate; Lane 2: COL3a1 knockout BXPC3 cell lysate; Predicted MW: 111,138kd Observed MW: 110kd Primary Ab: 1ug/ml Rabbit Anti-Human COL3a1 Antibody Second Ab: 0.2ug/mL HRP-Linked Caprine Anti-Rabbit IgG Polyclonal Antibody (#MBS2086047))

NCBI and Uniprot Product Information

• NCBI GI #: 494319369
• NCBI GeneID: 1281
• Molecular Weight: 111,907 Da
• NCBI Official Full Name: collagen type III alpha 1
• NCBI Official Synonym Full Names: collagen type III alpha 1 chain
• NCBI Official Symbol: COL3A1
• NCBI Official Synonym Symbols: EDS4A
• NCBI Protein Information: collagen alpha-1(III) chain
• UniProt Protein Name: Collagen alpha-1(III) chain
• Protein Family: Collagen
• UniProt Gene Name: COL3A1

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: Cell adhesion; Extracellular matrix; Motility/polarity/chemotaxis; Secreted; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 2q32.2

Cellular Component: collagen type III; endoplasmic reticulum lumen; extracellular matrix; extracellular region; extracellular space

Molecular Function: extracellular matrix structural constituent; integrin binding; platelet-derived growth factor binding; protein binding

Biological Process: cell-matrix adhesion; cerebral cortex development; collagen catabolic process; collagen fibril organization; extracellular matrix organization and biogenesis; fibril organization and biogenesis; heart development; integrin-mediated signaling pathway; negative regulation of immune response; peptide cross-linking; positive regulation of Rho protein signal transduction; regulation of immune response; response to cytokine stimulus; response to radiation; skin development; transforming growth factor beta receptor signaling pathway; wound healing

Disease: Ehlers-danlos Syndrome, Type Iii; Ehlers-danlos Syndrome, Type Iv, Autosomal Dominant