Reactome | ALDH4A1 oxidizes 1PYR-3OH-5COOH

ALDH4A1 oxidizes 1PYR-3OH-5COOH

Stable Identifier

R-HSA-9929439

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

Synonyms

(3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate + 2 H2O + NAD(+) => (4S)-4-hydroxy-L-glutamate + H(+) + NADH

ReviewStatus

5/5

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Mitochondrial ALDH4A1 catalyzes the reaction of 1PYR-3OH-5COOH (3R,5S)-1-pyrroline-3-hydroxy-5-carboxylate) and NAD+ to form 4-OH-L-Glu (erythro-4-hydroxy-L-glutamate) and NADH + H+. The reaction was first described by Adams & Goldstone (1960). Later studies by Valle et al. (1979) of hyperprolinemia patients identified ALDH4A1 as the catalyst of the reaction.

Literature References

PubMed ID	Title	Journal	Year
500817	Genetic evidence for a common enzyme catalyzing the second step in the degradation of proline and hydroxyproline Valle, D, Goodman, SI, Harris, SC, Phang, JM	J Clin Invest	1979
13681370	Hydroxyproline metabolism. IV. Enzymatic synthesis of gamma-hydroxyglutamate from Delta 1-pyrroline-3-hydroxy-5-carboxylate Adams, E, Goldstone, A	J Biol Chem	1960