L-2-hydroxyglutarate dehydrogenase: identification of a novel enzyme activity in rat and human liver. Implications for L-2-hydroxyglutaric acidemia.
In this paper we studied the degradation of L-2-hydroxyglutarate in tissues from rat and man in order to try and find the underlying basis for the accumulation of this metabolite in L-2-hydroxyglutaric acidemia patients. The results show that L-2-hydroxyglutarate is not degraded by an oxidase but via a dehydrogenase which was found to be present in liver only. This newly identified enzyme activity was characterized kinetically, although the nature of the reaction product remains to be identified.
Jansen
G A
GA
Department of Paediatrics and Clinical Biochemistry, University Hospital Amsterdam, Academic Medical Centre, The Netherlands.
Wanders
R J
RJ
D000429:Alcohol Oxidoreductases / Q000737:chemistry / Q000172:deficiency / Q000378:metabolism*; D000818:Animals; D005977:Glutarates / Q000097:blood*; D006801:Humans; D007700:Kinetics; D008099:Liver / Q000201:enzymology*; D051381:Rats
D005977:Glutarates; C019417:alpha-hydroxyglutarate; D000429:Alcohol Oxidoreductases; C023858:2-hydroxyglutarate dehydrogenase