Reactome | PRODH2 oxidises HPRO to 1PYR-5COOH

PRODH2 oxidises HPRO to 1PYR-5COOH

Stable Identifier

R-HSA-8955817

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix, mitochondrial inner membrane

Locations in the PathwayBrowser

Expand all

General

SBML | | PDF

SVG | | PPTX | SBGN

Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

The catabolism of trans-4-hydroxy-L-proline (HPRO) and proline (L-Pro) (from endogenous and dietary sources of collagen) makes a significant contribution to the glyoxylate pool in humans. The dehydrogenation (oxidation) of HPRO/L-Pro to L-1-pyrroline-5-carboxylate (1PYR-5COOH) coupled to the conversion of FAD to FADH2 is the first step in proline catabolism. Proline dehydrogenases (PRODHs), located at the inner mitochondrial membrane mediate this reaction. Whereas PRODH prefers L-Pro as substrate, PRODH2 has a clear preference for HPRO (Summitt et al. 2015).

Literature References

PubMed ID	Title	Journal	Year
25697095	Proline dehydrogenase 2 (PRODH2) is a hydroxyproline dehydrogenase (HYPDH) and molecular target for treating primary hyperoxaluria Summitt, CB, Johnson, LC, Jönsson, TJ, Parsonage, D, Holmes, RP, Lowther, WT	Biochem. J.	2015