Recombinant Human Prolyl 3-hydroxylase 3 (LEPREL2)

Uniprot Id

Q8IVL6

Target Species

Human

Target Name

P3H3

Target Full Name

Prolyl 3-hydroxylase 3

Target Function

Part of a complex composed of PLOD1, P3H3 and P3H4 that catalyzes hydroxylation of lysine residues in collagen alpha chains and is required for normal assembly and cross-linkling of collagen fibrils. Required for normal hydroxylation of lysine residues in type I collagen chains in skin, bone, tendon, aorta and cornea. Required for normal skin stability via its role in hydroxylation of lysine residues in collagen alpha chains and in collagen fibril assembly. Apparently not required for normal prolyl 3-hydroxylation on collagen chains, possibly because it functions redundantly with other prolyl 3-hydroxylases.

Target Subcellular Location

Endoplasmic reticulum.

Target Protein Families

Leprecan family

Target Tissue Specificity

Detected in fetal cartilage (at protein level). Weak expression in heart, lung, ovary and skeletal muscle.

Target Synonyms

P3H3; LEPREL2; Prolyl 3-hydroxylase 3; EC 1.14.11.7; Leprecan-like protein 2; Protein B

Target Background

The protein encoded by this gene belongs to the leprecan family of proteoglycans, which function as collagen prolyl hydroxylases that are required for proper collagen biosynthesis, folding and assembly. This protein, like other family members, is thought to reside in the endoplasmic reticulum. Epigenetic inactivation of this gene is associated with breast and other cancers, suggesting that it may function as a tumor suppressor.