Glutamate dehydrogenase 1, mitochondrial

Definition:

Mitochondrial glutamate dehydrogenase that catalyzes the conversion of L-glutamate into alpha-ketoglutarate. Plays a key role in glutamine anaplerosis by producing alpha-ketoglutarate, an important intermediate in the tricarboxylic acid cycle. Plays a role in insulin homeostasis. May be involved in learning and memory reactions by increasing the turnover of the excitatory neurotransmitter glutamate (By similarity).

References:

[1]. Myung-Min Choi, et al. Identification of ADP-ribosylation site in human glutamate dehydrogenase isozymes. FEBS Lett. 2005 Aug 1;579(19):4125-30. [Content Brief]

[2]. T J Smith, et al. Structures of bovine glutamate dehydrogenase complexes elucidate the mechanism of purine regulation. J Mol Biol. 2001 Mar 23;307(2):707-20. [Content Brief]

[3]. Marcia C Haigis, et al. SIRT4 inhibits glutamate dehydrogenase and opposes the effects of calorie restriction in pancreatic beta cells. Cell. 2006 Sep 8;126(5):941-54. [Content Brief]

[4]. C A Stanley, et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med. 1998 May 7;338(19):1352-7. [Content Brief]

[5]. A Plaitakis, et al. Nerve tissue-specific (GLUD2) and housekeeping (GLUD1) human glutamate dehydrogenases are regulated by distinct allosteric mechanisms: implications for biologic function. J Neurochem. 2000 Nov;75(5):1862-9. [Content Brief]

[6]. C MacMullen, et al. Hyperinsulinism/hyperammonemia syndrome in children with regulatory mutations in the inhibitory guanosine triphosphate-binding domain of glutamate dehydrogenase. J Clin Endocrinol Metab. 2001 Apr;86(4):1782-7. [Content Brief]

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