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MGP006926

UniProt Annotations

Entry Information
Gene Nameprolyl 4-hydroxylase, alpha polypeptide III
Protein EntryP4HA3_HUMAN
UniProt IDQ7Z4N8
SpeciesHuman
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=3; Name=1; IsoId=Q7Z4N8-1; Sequence=Displayed; Name=2; IsoId=Q7Z4N8-2; Sequence=VSP_031146, VSP_031147; Note=No experimental confirmation available.; Name=3; IsoId=Q7Z4N8-3; Sequence=VSP_054131; Note=No experimental confirmation available.;
Catalytic ActivityL-proline-[procollagen] + 2-oxoglutarate + O(2) = trans-4-hydroxy-L-proline-[procollagen] + succinate + CO(2).
CofactorName=Fe(2+); Xref=ChEBI:CHEBI:29033; Note=Binds 1 Fe(2+) ion per subunit.;
CofactorName=L-ascorbate; Xref=ChEBI:CHEBI:38290;
FunctionCatalyzes the post-translational formation of 4- hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins. {ECO:0000269|PubMed:12874193, ECO:0000269|PubMed:14500733}.
PtmN-glycosylation plays no role in the catalytic activity.
SimilarityBelongs to the P4HA family. {ECO:0000305}.
SimilarityContains 1 Fe2OG dioxygenase domain. {ECO:0000255|PROSITE-ProRule:PRU00805}.
SimilarityContains 1 TPR repeat. {ECO:0000305}.
Subcellular LocationEndoplasmic reticulum lumen {ECO:0000305}.
SubunitHeterotetramer of two alpha-3 chains and two beta chains (the beta chain is the multi-functional PDI). {ECO:0000269|PubMed:12874193, ECO:0000269|PubMed:14500733}.
Tissue SpecificityHighly expressed in placenta, liver and fetal skin. Weakly expressed in fetal epiphyseal cartilage, fetal liver, fibroblast, lung and skeletal muscle. Expressed also in fibrous cap of carotid atherosclerotic lesions. {ECO:0000269|PubMed:12874193, ECO:0000269|PubMed:14500733}.
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