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. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014],
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Function:
Catalytic activity:Procollagen L-proline + 2-oxoglutarate + O(2) = procollagen trans-4-hydroxy-L-proline + succinate + CO(2).,cofactor:Ascorbate.,cofactor:Binds 1 Fe(2+) ion per subunit.,Function:Catalyzes the post-translational formation of 4-hydroxyproline in -Xaa-Pro-Gly- sequences in collagens and other proteins.,PTM:N-glycosylation plays no role in the catalytic activity.,similarity:Belongs to the P4HA family.,similarity:Contains 1 PKHD (prolyl/lysyl hydroxylase) domain.,similarity:Contains 1 TPR repeat.,subunit:Heterotetramer of two alpha-3 chains and two beta chains (the beta chain is the multi-functional PDI).,tissue specificity:Highly 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.,
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Cellular Localization:
Endoplasmic reticulum lumen .
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Tissue Expression:
Highly 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.
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Research Areas:
>>Arginine and proline metabolism ;
>>Metabolic pathways