COL3A1 blocking peptide

COL3A1 Peptide - N-terminal region

Cat. Num. AAA3246241

• Gene Names: COL3A1; EDS4A; EDSVASC
• Reactivity: Human
• Synonyms: COL3A1; COL3A1 Peptide - N-terminal region; COL3A1 blocking peptide

• Reactivity: Human
• Form/Format: Lyophilized powder
• Sequence: Synthetic peptide located within the following region: CDDQELDCPNPEIPFGECCAVCPQPPTAPTRPPNGQGPQGPKGDPGPPGI
• Sequence Length: 1466

• Preparation and Storage: Add 100ul of sterile PBS. Final peptide concentration is 1 mg/ml in PBS. For longer periods of storage, store at -20 degree C. Avoid repeat freeze-thaw cycles.

Related Product Information for COL3A1 blocking peptide

This is a synthetic peptide designed for use in combination with anti- COL3A1 Antibody, made

Target 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.

Product Categories/Family for COL3A1 blocking peptide

Peptide

NCBI and Uniprot Product Information

• NCBI GI #: 4502951
• NCBI GeneID: 1281
• NCBI Accession #: NP_000081.1
• NCBI GenBank Nucleotide #: NM_000090.3
• UniProt Accession #: P02461
• Molecular Weight: 161 kDa
• NCBI Official Full Name: collagen alpha-1(III) chain preproprotein
• NCBI Official Synonym Full Names: collagen type III alpha 1 chain
• NCBI Official Symbol: COL3A1
• NCBI Official Synonym Symbols: EDS4A; EDSVASC
• NCBI Protein Information: collagen alpha-1(III) chain
• UniProt Protein Name: Collagen alpha-1(III) chain
• Protein Family: Collagen
• UniProt Gene Name: COL3A1
• UniProt Entry Name: CO3A1_HUMAN

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