P4HA1

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 encoding different isoforms have been described. [provided by RefSeq, Jul 2008]

Prolyl 4-Hydroxylase Subunit Alpha 1

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

Collagen fibril organizationIEA:Ensembl
Peptidyl-proline hydroxylation to 4-hydroxy-L-prolineManual Assertion Based On ExperimentIBA:GO_Central

Endoplasmic reticulum lumen