Glutaric aciduria type I: pathomechanisms of neurodegeneration

B. Flott-Rahmel; P. Schluff; U. Musshoff; E.A. Das-Smaal; T. Lucke; R. Steinfeld; E. Christensen; C.A.J.M. Jakobs; A. Ludolph; A. Neu; R. Roper; K. Ullrich

doi:10.1023/A:1005595921323

Glutaric aciduria type I: pathomechanisms of neurodegeneration

B. Flott-Rahmel, P. Schluff, U. Musshoff, E.A. Das-Smaal, T. Lucke, R. Steinfeld, E. Christensen, C.A.J.M. Jakobs, A. Ludolph, A. Neu, R. Roper, K. Ullrich

Cognitive Psychology

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Abstract

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 Mg

Original language	English
Pages (from-to)	392-403
Journal	Journal of Inherited Metabolic Disease
Volume	22
DOIs	https://doi.org/10.1023/A:1005595921323
Publication status	Published - 1999

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title = "Glutaric aciduria type I: pathomechanisms of neurodegeneration",

abstract = "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 Mg",

author = "B. Flott-Rahmel and P. Schluff and U. Musshoff and E.A. Das-Smaal and T. Lucke and R. Steinfeld and E. Christensen and C.A.J.M. Jakobs and A. Ludolph and A. Neu and R. Roper and K. Ullrich",

year = "1999",

doi = "10.1023/A:1005595921323",

language = "English",

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TY - JOUR

T1 - Glutaric aciduria type I: pathomechanisms of neurodegeneration

AU - Flott-Rahmel, B.

AU - Schluff, P.

AU - Musshoff, U.

AU - Das-Smaal, E.A.

AU - Lucke, T.

AU - Steinfeld, R.

AU - Christensen, E.

AU - Jakobs, C.A.J.M.

AU - Ludolph, A.

AU - Neu, A.

AU - Roper, R.

AU - Ullrich, K.

PY - 1999

Y1 - 1999

N2 - 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 Mg

AB - 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 Mg

U2 - 10.1023/A:1005595921323

DO - 10.1023/A:1005595921323

M3 - Article

SN - 0141-8955

VL - 22

SP - 392

EP - 403

JO - Journal of Inherited Metabolic Disease

JF - Journal of Inherited Metabolic Disease

ER -

Glutaric aciduria type I: pathomechanisms of neurodegeneration

Abstract

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