prolyl 4-hydroxylase, alpha polypeptide I
Symbol
P4HA1
(may also be known as: None)
Organism
Human
Description
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]
Orthologs
| Symbol | Species | |
|---|---|---|
| p4ha1b | Danio rerio | |
| P4ha1 | Mus musculus | |
| P4ha1 | Rattus norvegicus |
Links to external resources
Changes associated with this gene
GO Terms
| GO ID | GO Term | GO Category | |
|---|---|---|---|
| GO:0018401 | peptidyl-proline hydroxylation to 4-hydroxy-L-proline | biological_process | |
| GO:0030199 | collagen fibril organization | biological_process | |
| GO:0005739 | mitochondrion | cellular_component | |
| GO:0005783 | endoplasmic reticulum | cellular_component | |
| GO:0005788 | endoplasmic reticulum lumen | cellular_component | |
| GO:0043231 | intracellular membrane-bounded organelle | cellular_component | |
| GO:0004656 | procollagen-proline 4-dioxygenase activity | molecular_function | |
| GO:0005488 | binding | molecular_function | |
| GO:0005506 | iron ion binding | molecular_function | |
| GO:0016702 | oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen | molecular_function | |
| GO:0016706 | oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors | molecular_function | |
| GO:0031418 | L-ascorbic acid binding | molecular_function | |
| GO:0046872 | metal ion binding | molecular_function | |