PLOD3
Description
The PLOD3 (procollagen-lysine,2-oxoglutarate 5-dioxygenase 3) is a protein-coding gene located on chromosome 7.
PLOD3, also known as procollagen-lysine,2-oxoglutarate 5-dioxygenase 3, is an enzyme encoded by the PLOD3 gene. It functions as a membrane-bound homodimer located in the cisternae of the rough endoplasmic reticulum. PLOD3 uses iron and ascorbate as cofactors to catalyze the hydroxylation of lysine residues in collagen-like peptides. These hydroxylated residues serve as attachment points for carbohydrates within collagen, contributing to the stability of intermolecular crosslinks. Defects in lysyl hydroxylase activity, such as those found in some patients with Ehlers-Danlos syndrome type VIB, can lead to various connective tissue disorders.
PLOD3 is a multifunctional enzyme that performs essential post-translational modifications on lysine residues within procollagen. It plays a role in forming hydroxylysine residues in specific collagen sequences (-Xaa-Lys-Gly-), transferring galactose to hydroxylysine to create galactosyl 5-hydroxylysine, and then adding glucose moieties to form 1,2-glucosylgalactosyl-5-hydroxylysine residues. PLOD3 also participates in the hydroxylation and glycosylation of lysine residues in the MBL1 collagen-like domain, leading to the formation of hydroxylysine and 1,2-glucosylgalactosyl-5-hydroxylysine residues. This enzyme is crucial for the proper biosynthesis and secretion of type IV collagens, contributing to the normal formation of basement membranes.
PLOD3 is also known as BCARD, LH3.
