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

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

mitochondrial matrix

Synonyms

4-OH-L-glutamate semialdehyde + NAD+ => 4-OH-L-glutamate + HADH + H+

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

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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