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prolyl 4-hydroxylase alpha 2; 4-PH alpha-2; procollagen-proline-2-oxoglutarate-4-dioxygenase alpha-2 subunit (P4HA2, P4HA)
Function:
- catalyzes post-translational formation of 4-hydroxyproline in -Xaa-Pro-gly- sequences in collagens & other proteins
procollagen L-proline + 2-oxoglutarate + O2
procollagen trans-4-hydroxy-L-proline + succinate + CO2
Structure:
- heterotetramer of two alpha-2 chains & two beta chains (the beta chain is the multi-functional PDI)
- belongs to the P4HA family
- contains 1 PKHD (prolyl/lysyl hydroxylase) domain
- contains 1 TPR repeat
Compartment: endoplasmic reticulum lumen
Alternative splicing: named isoforms=2
General
glycoprotein
prolyl 4-hydroxylase alpha subunit
Properties
SIZE: entity length = 535 aa
MW = 61 kD
COMPARTMENT: endoplasmic reticulum
MOTIF: signal sequence {1-21}
N-glycosylation site {N115}
tetratricopeptide repeat {207-240}
N-glycosylation site {N264}
PKHD {336-519}
MOTIF: Iron [Fe]-binding site
SITE: 430-430
Iron [Fe]-binding site
SITE: 432-432
Iron [Fe]-binding site
SITE: 501-501
lysine residue {K511}
Database Correlations
OMIM 600608
UniProt O15460
PFAM correlations
Kegg hsa:8974
ENZYME 1.14.11.2
References
- UniProt :accession P13674
- UniProt :accession O15460