Glutaric aciduria type I:: Pathomechanisms of neurodegeneration

被引：84

作者：

Ullrich, K

Flott-Rahmel, B

Schluff, P

Musshoff, U

Das, A

Lücke, T

Steinfeld, R

Christensen, E

Jakobs, C

Ludolph, A

Neu, A

Röper, R

机构：

[1] Univ Hamburg, Klin & Poliklin Kinder & Jugendmed, Dept Pediat, D-20246 Hamburg, Germany

[2] Univ Munster, Dept Pediat, D-4400 Munster, Germany

[3] Univ Munster, Inst Physiol, D-4400 Munster, Germany

[4] Univ Copenhagen, Dept Pediat, DK-1168 Copenhagen, Denmark

[5] Free Univ Amsterdam, Dept Clin Chem, NL-1081 HV Amsterdam, Netherlands

[6] Univ Ulm, Dept Neurol, D-89069 Ulm, Germany

[7] Univ Hamburg, Dept Mol Neurobiol, D-20246 Hamburg, Germany

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1999年 / 22卷 / 04期

关键词：

D O I：

10.1023/A:1005595921323

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

In organotypic corticostriatal and hippocampal slice cultures from rat brain, 3-hydroxyglutaric acid but not glutaric and glutaconic acids induced neurodegeneration by activation of NMDA receptors. Electrophysiological investigations (Xenopus laevis oocytes expressing glutamate receptors; rat mixed cortex culture) revealed no direct interaction of 3-hydroxyglutaric acid with glutamate receptors. We speculate that 3-hydroxyglutaric acid induces a mild energy deprivation that interferes with the voltage-dependent Mg2+-block of NMDA receptors.

引用

页码：392 / 403

页数：12

共 42 条

[21] GLUTARIC ACIDEMIA - A METABOLIC DISORDER CAUSING PROGRESSIVE CHOREOATHETOSIS [J].