IUBMB Enzyme Nomenclature


Accepted name: hydroxyproline dehydrogenase

Reaction: trans-4-hydroxy-L-proline + a quinone = (3R,5S)-3-hydroxy-1-pyrroline-5-carboxylate + a quinol

Other name(s): HYPDH; OH-POX; hydroxyproline oxidase; PRODH2 (gene name)

Systematic name: trans-4-hydroxy-L-proline:quinone oxidoreductase

Comments: A flavoprotein (FAD). The enzyme from human also has low activity with L-proline (cf. EC, proline dehydrogenase).

Links to other databases: BRENDA, EXPASY, KEGG, MetaCyc, CAS registry number:


1. Cooper, S.K., Pandhare, J., Donald, S.P. and Phang, J.M. A novel function for hydroxyproline oxidase in apoptosis through generation of reactive oxygen species. J. Biol. Chem. 283 (2008) 10485-10492. [PMID: 18287100]

2. Summitt, C.B., Johnson, L.C., Jonsson, T.J., Parsonage, D., Holmes, R.P. and Lowther, W.T. Proline dehydrogenase 2 (PRODH2) is a hydroxyproline dehydrogenase (HYPDH) and molecular target for treating primary hyperoxaluria. Biochem. J. 466 (2015) 273-281. [PMID: 25697095]

[EC created 2017]

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