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]
Function:
Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.
Subcellular Location:
Endoplasmic reticulum lumen.
Similarity:
Belongs to the P4HA family.
Contains 1 Fe2OG dioxygenase domain.
Contains 1 TPR repeat.
SWISS:
P13674
Gene ID:
5033
Database links:
Entrez Gene: 5033 Human
Entrez Gene: 18451 Mouse
Entrez Gene: 64475 Rat
SwissProt: P13674 Human
SwissProt: Q60715 Mouse
SwissProt: P54001 Rat
Unigene: 500047 Human
Unigene: 593005 Human
Unigene: 2212 Mouse
Unigene: 8531 Rat
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