L-2-HYDROXYGLUTARATE DEHYDROGENASE - IDENTIFICATION OF A NOVEL ENZYME-ACTIVITY IN RAT AND HUMAN LIVER - IMPLICATIONS FOR L-2-HYDROXYGLUTARIC ACIDEMIA

Citation
Ga. Jansen et Rja. Wanders, L-2-HYDROXYGLUTARATE DEHYDROGENASE - IDENTIFICATION OF A NOVEL ENZYME-ACTIVITY IN RAT AND HUMAN LIVER - IMPLICATIONS FOR L-2-HYDROXYGLUTARIC ACIDEMIA, Biochimica et biophysica acta, 1225(1), 1993, pp. 53-56
Citations number
15
Language
INGLESE
art.tipo
Article
Categorie Soggetti
Biophysics,Biology
ISSN journal
0006-3002
Volume
1225
Issue
1
Year of publication
1993
Pages
53 - 56
Database
ISI
SICI code
0006-3002(1993)1225:1<53:LD-IOA>2.0.ZU;2-G
Abstract
In this paper we studied the degradation of L-2-hydroxyglutarate in ti ssues from rat and man in order to try and find the underlying basis f or 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 k inetically, although the nature of the reaction product remains to be identified.