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

Stable Identifier
R-HSA-6784399
Type
Reaction [transition]
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
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
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
22516612 The three-dimensional structural basis of type II hyperprolinemia

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

J. Mol. Biol. 2012
Participants
Participant Of
Event Information
Catalyst Activity
Catalyst Activity
Title
1-pyrroline-5-carboxylate dehydrogenase activity of ALDH4A1 dimer [mitochondrial matrix]
Physical Entity
Activity
Orthologous Events
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