CATH Classification

Domain Context

CATH Clusters

Superfamily Tetratricopeptide repeat domain
Functional Family Prolyl 4-hydroxylase subunit alpha 2

Enzyme Information

1.14.11.2
Procollagen-proline 4-dioxygenase.
based on mapping to UniProt P13674
L-proline-[procollagen] + 2-oxoglutarate + O(2) = trans-4-hydroxy-L- proline-[procollagen] + succinate + CO(2).
-!- The enzyme, which is located within the lumen of the endoplasmic reticulum, catalyzes the 4-hydroxylation of prolines in -X-Pro-Gly- sequences. -!- The 4-hydroxyproline residues are essential for the formation of the collagen triple helix. -!- The enzyme forms a complex with protein disulfide isomerase and acts not only on procollagen but also on more than 15 other proteins that have collagen-like domains.

UniProtKB Entries (1)

P13674
P4HA1_HUMAN
Homo sapiens
Prolyl 4-hydroxylase subunit alpha-1

PDB Structure

PDB 4BT8
External Links
Method X-RAY DIFFRACTION
Organism
Primary Citation
The Structural Motifs for Substrate Binding and Dimerization of the Alpha Subunit of Collagen Prolyl 4-Hydroxylase
Anantharajan, J., Koski, M.K., Kursula, P., Hieta, R., Bergmann, U., Myllyharju, J., Wierenga, R.K.
Structure