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

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
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