P4HA3

This gene encodes a component of prolyl 4-hydroxylase, a key enzyme in collagen synthesis composed of two identical alpha subunits and two beta subunits. The encoded protein is one of several different types of alpha subunits and provides the major part of the catalytic site of the active enzyme. In collagen and related proteins, prolyl 4-hydroxylase catalyzes the formation of 4-hydroxyproline that is essential to the proper three-dimensional folding of newly synthesized procollagen chains. Alternatively spliced transcript variants have been observed, but their full-length nature has not been determined. [provided by RefSeq]

Full Name

prolyl 4-hydroxylase, alpha polypeptide III

Function

Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.

Biological Process

Peptidyl-proline hydroxylation to 4-hydroxy-L-prolineManual Assertion Based On ExperimentIBA:GO_Central

Cellular Location

Endoplasmic reticulum lumen

PTM

N-glycosylation plays no role in the catalytic activity.