prolyl 4-hydroxylase alpha 1; 4-PH alpha-1; procollagen-proline-2-oxoglutarate-4-dioxygenase alpha-1 subunit (P4HA1)

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

Cofactor: iron, ascorbate

Structure

• heterotetramer of two alpha-1 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

More general terms

• prolyl 4-hydroxylase alpha subunit

Database

• UniProt: http://www.uniprot.org/uniprot/P13674.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:5033
• OMIM: https://mirror.omim.org/entry/176710