Reactome | PRODH oxidises L-Pro to 1PYR-5COOH

PRODH oxidises L-Pro to 1PYR-5COOH

Stable Identifier

R-HSA-70670

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix, mitochondrial inner membrane

Synonyms

proline => L-1-pyrroline-5-carboxylate

ReviewStatus

5/5

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The dehydrogenation (oxidation) of proline to L-1-pyrroline-5-carboxylate coupled to the conversion of FAD to FADH2 is the first step in proline breakdown (Bender et al. 2005). Proline dehydrogenase (PRODH), the enzyme that catalyzes this reaction, is found in liver, kidney, and brain cells, where it is tightly bound to the inner mitochondrial membrane.

Literature References

PubMed ID	Title	Journal	Year
15662599	Functional consequences of PRODH missense mutations Lin, WW, Willis, A, Valle, D, Pulver, A, Bender, HU, Steel, G, Hu, CA, Almashanu, S	Am J Hum Genet	2005