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

被引：27

作者：

JANSEN, GA ^{[1
]}

WANDERS, RJA ^{[1
]}

机构：

[1] UNIV AMSTERDAM HOSP,ACAD MED CTR,DEPT PAEDIAT & CLIN BIOCHEM,1105 AZ AMSTERDAM,NETHERLANDS

来源：

BIOCHIMICA ET BIOPHYSICA ACTA | 1993年 / 1225卷 / 01期

关键词：

L-2-HYDROXYGLUTARATE; L-2-HYDROXYGLUTARATE DEHYDROGENASE; L-2-HYDROXYGLUTARIC ACIDEMIA; ENZYME ACTIVITY; ENZYME DEGRADATION; (LIVER); (RAT); (HUMAN);

D O I：

10.1016/0925-4439(93)90121-G

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：53 / 56

页数：4

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