Prolyl 4-Hydroxylase Subunit Alpha-1 (P4HA1) ELISA Kit for Human

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]

P4HA1: Catalyzes the post-translational formation of 4- hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins. Belongs to the P4HA family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Endoplasmic reticulum; Amino Acid Metabolism - arginine and proline; EC 1.14.11.2; Oxidoreductase; Mitochondrial

Chromosomal Location of Human Ortholog: 10q22.1

Cellular Component: endoplasmic reticulum; endoplasmic reticulum lumen; intracellular membrane-bound organelle; membrane; mitochondrion

Molecular Function: iron ion binding; L-ascorbic acid binding; oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen; procollagen-proline 4-dioxygenase activity; protein binding

Biological Process: collagen fibril organization; peptidyl-proline hydroxylation to 4-hydroxy-L-proline