Reactome | alpha-ketoglutarate + NH4+ + NAD(P)H + H+ <=> glutamate + NAD(P)+ [GLUD1]

alpha-ketoglutarate + NH4+ + NAD(P)H + H+ <=> glutamate + NAD(P)+ [GLUD1]

Stable Identifier

R-HSA-70589

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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alpha-ketoglutarate + NH4+ + NAD(P)H + H+ <=> glutamate + NAD(P)+ [GLUD1]

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Mitochondrial glutamate dehydrogenase 1 (GLUD1) catalyzes the reversible reaction of 2-oxoglutarate, NAD(P)H + H+, and ammonia to form glutamate and NAD(P)+ (Fang et al. 2002). Mature GLUD1 protein lacks the 53 aminoterminal residues of the nascent protein (Julliard and Smith 1979), which function as a mitochondrial import signal. The active form of the enzyme is a hexamer, allosterically activated by ADP and inhibited by GTP (Fang et al. 2002; Smith et al. 2002).

Literature References

PubMed ID	Title	Journal	Year
429360	Partial amino acid sequence of the glutamate dehydrogenase of human liver and a revision of the sequence of the bovine enzyme Julliard, JH, Smith, EL	J Biol Chem	1979
11903050	Expression, purification and characterization of human glutamate dehydrogenase (GDH) allosteric regulatory mutations Smith, TJ, MacMullen, CM, Hsu, BY, Fang, J, Poncz, M, Stanley, CA	Biochem J	2002
12054821	The structure of apo human glutamate dehydrogenase details subunit communication and allostery Smith, TJ, Wu, J, Schmidt, T, Fang, J, Stanley, CA, Siuzdak, G	J Mol Biol	2002