ELK Biotechnology Collagen V α1 (Cleaved-Ala1605) rabbit pAb

Collagen V α1 (Cleaved-Ala1605) Rabbit pAb

제품 정보

Background

Defects in COL5A1 are associated with Ehlers-Danlos syndrome type 1 (EDS1) [MIM:130000], also known as Ehlers-Danlos syndrome gravis or severe classic type, and type 2 (EDS2) [MIM:130010], known as mild classic type.
EDS is a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility, and joint hyperlaxity.

Function:
Type V collagen is a member of group I collagen (fibrillar forming collagen). It is a minor connective tissue component with nearly ubiquitous distribution. Type V collagen binds to DNA, heparan sulfate, thrombospondin, heparin, and insulin.

Post-translational Modifications (PTM):

• Hydroxylation of prolines at the third position of the tripeptide repeating unit (G-X-Y) in some or all chains
• Sulfation on 40% of tyrosines

Similarity:

• Belongs to the fibrillar collagen family
• Contains 1 laminin G-like domain
• Contains 1 TSP N-terminal (TSPN) domain

Subunit Structure:
Trimers composed of two alpha 1(V) and one alpha 2(V) chains in most tissues, and trimers of one alpha 1(V), one alpha 2(V), and one alpha 3(V) chains in placenta. Interacts with CSPG4.