ALDH4A1 dimer dehydrogenates 4-OH-L-glutamate semialdehyde to 4-OH-L-glutamate

Stable Identifier
R-HSA-6784399
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
4-OH-L-glutamate semialdehyde + NAD+ => 4-OH-L-glutamate + HADH + H+
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Mitochondrial delta-1-pyrroline-5-carboxylate dehydrogenase (ALDH4A1) catalyzes the reaction of 4-hydroxy-L-glutamate gamma-semialdehyde and NAD+ to form 4-hydroxyglutamate and NADH + H+. ALDH4A1 also catalyzes the corresponding reaction of proline catabolism, as shown in biochemical and structural studies (Adams & Goldstone 1960; Srivastava et al. 2012), and mutations in ALDH4A1 disrupt both catabolic processes in human patients (Valle et al. 1979).

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, Phang, JM, Goodman, SI, Harris, SC

J Clin Invest 1979
13681370 Hydroxyproline metabolism. IV. Enzymatic synthesis of gamma-hydroxyglutamate from Delta 1-pyrroline-3-hydroxy-5-carboxylate

Goldstone, A, Adams, E

J Biol Chem 1960
22516612 The three-dimensional structural basis of type II hyperprolinemia

Tanner, JJ, Singh, RK, Becker, DF, Moxley, MA, Srivastava, D, Henzl, MT

J. Mol. Biol. 2012
Participants
Participates
Event Information
Catalyst Activity

1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]

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