Entrez Summary: 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].
UniProt Summary: Catalyzes the post-translational formation of 4- hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Pfam DomainsGO Terms
Pfam Domains
2OG-FeII Oxy
P4Ha N
GO Terms
procollagen-proline 4-dioxygenase activity
peptidyl-proline hydroxylation to 4-hydroxy-L-proline
oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen
peptidyl-proline hydroxylation
4-hydroxyproline metabolic process
L-ascorbic acid binding
protein hydroxylation
peptidyl-proline modification
electron transfer activity
iron ion binding
electron transport chain
cellular modified amino acid metabolic process
alpha-amino acid metabolic process
endoplasmic reticulum lumen
cellular amino acid metabolic process
generation of precursor metabolites and energy
intracellular membrane-bounded organelle
peptidyl-amino acid modification
carboxylic acid metabolic process
oxidation-reduction process
oxoacid metabolic process
endoplasmic reticulum
organic acid metabolic process
small molecule metabolic process
CRISPR Data
Compound HitMost Correlated Genes in ChemogenomicsTissues where Essential in the Avana Dataset (DepMap 20Q1)