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

收藏

页码：392 / 403

页数：12

相关论文

共 42 条

[1] NMDA RECEPTOR LEVELS IN CHRONICALLY DEPOLARIZED LONG-TERM NEONATAL RAT NEOCORTICAL EXPLANTS [J].

BAKER, RE ;

VERMEULEN, JP ;

BOER, GJ .

INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE, 1992, 10 (05) :413-419

[2] AMINO-ACIDS AS CENTRAL NERVOUS TRANSMITTERS - INFLUENCE OF IONS, AMINO-ACID ANALOGS, AND ONTOGENY ON TRANSPORT-SYSTEMS FOR L-GLUTAMIC AND L-ASPARTIC ACIDS AND GLYCINE INTO CENTRAL NERVOUS SYNAPTOSOMES OF RAT [J].

BENNETT, JP ;

LOGAN, WJ ;

SNYDER, SH .

JOURNAL OF NEUROCHEMISTRY, 1973, 21 (06) :1533-1550

[3] Glutamate in neurologic diseases [J].

Bittigau, P ;

Ikonomidou, C .

JOURNAL OF CHILD NEUROLOGY, 1997, 12 (08) :471-485

[4] IMPROVED ASSAY OF GLUTARYL-COA DEHYDROGENASE IN CULTURED-CELLS AND LIVER - APPLICATION TO GLUTARIC ACIDURIA TYPE-I [J].

CHRISTENSEN, E .

CLINICA CHIMICA ACTA, 1983, 129 (01) :91-97

[5] Regulation of mitochondrial ATP synthase activity in human myocardium [J].

Das, AM .

CLINICAL SCIENCE, 1998, 94 (05) :499-504

[6] GLUTAMATE AND GAMMA-AMINOBUTYRIC-ACID NEUROTRANSMITTER SYSTEMS IN THE ACUTE PHASE OF MAPLE SYRUP URINE DISEASE AND CITRULLINEMIA ENCEPHALOPATHIES IN NEWBORN CALVES [J].

DODD, PR ;

WILLIAMS, SH ;

GUNDLACH, AL ;

HARPER, PAW ;

HEALY, PJ ;

DENNIS, JA ;

JOHNSTON, GAR .

JOURNAL OF NEUROCHEMISTRY, 1992, 59 (02) :582-590

[7] Homocysteic and homocysteine sulphinic acid exhibit excitotoxicity in organotypic cultures from rat brain [J].

Flott-Rahmel, B ;

Schurmann, M ;

Schluff, P ;

Fingerhut, R ;

Musshoff, U ;

Fowler, B ;

Ullrich, K .

EUROPEAN JOURNAL OF PEDIATRICS, 1998, 157 (Suppl 2) :S112-S117

[8] Nerve cell lesions caused by 3-hydroxyglutaric acid: A possible mechanism for neurodegeneration in glutaric acidaemia I [J].

FlottRahmel, B ;

Falter, C ;

Schluff, P ;

Fingerhut, R ;

Christensen, E ;

Jakobs, C ;

Musshoff, U ;

Fautek, JD ;

Deufel, T ;

Ludolph, A ;

Ullrich, K .

JOURNAL OF INHERITED METABOLIC DISEASE, 1997, 20 (03) :387-390

[9] FORMATION OF LAYER-SPECIFIC FIBER PROJECTIONS TO THE HIPPOCAMPUS IN-VITRO [J].

FROTSCHER, M ;

HEIMRICH, B .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1993, 90 (21) :10400-10403

[10] ORGANOTYPIC MONOLAYER-CULTURES OF NERVOUS-TISSUE [J].

GAHWILER, BH .

JOURNAL OF NEUROSCIENCE METHODS, 1981, 4 (04) :329-342

← 1 2 3 4 5 →