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Biochim Biophys Acta. 1993 Nov 25;1225(1):53-6.

L-2-hydroxyglutarate dehydrogenase: identification of a novel enzyme activity in rat and human liver. Implications for L-2-hydroxyglutaric acidemia.

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Department of Paediatrics and Clinical Biochemistry, University Hospital Amsterdam, Academic Medical Centre, The Netherlands.


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.

[Indexed for MEDLINE]

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