Nerve cell lesions caused by 3-hydroxyglutaric acid: A possible mechanism for neurodegeneration in glutaric acidaemia I

Journal of Inherited Metabolic Disease

Volumn 20, Issue 3, 1997, Pages 387-390

  Flott Rahmel, B ^a,f   Falter, C ^a   Schluff, P ^b   Fingerhut, R ^b   Christensen, E ^c   Jakobs, C ^d   Musshoff, U ^a   Fautek, J D ^a   Deufel, T ^a   Ludolph, A ^e   Ullrich, K ^b  

^a UNIVERSITY OF MÜNSTER   (Germany)

^b UNIVERSITY OF HAMBURG   (Germany)

^c UNIVERSITY OF COPENHAGEN   (Denmark)

^d VU UNIVERSITY AMSTERDAM   (Netherlands)

^e HUMBOLDT UNIVERSITY   (Germany)

^f UNIVERSITY HOSPITAL MÜNSTER   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

EXCITOTOXIN; GLUTAMATE RECEPTOR; GLUTAMATE RECEPTOR AGONIST; GLUTARIC ACID; GLUTARIC ACID DERIVATIVE;

ACIDEMIA; ANIMAL CELL; ANIMAL TISSUE; BRAIN ELECTROPHYSIOLOGY; BRAIN METABOLISM; CONFERENCE PAPER; CONTROLLED STUDY; HIPPOCAMPUS; NERVE CELL LESION; NERVE DEGENERATION; NEUROTOXICITY; NONHUMAN; OOCYTE; RAT;

ADENOSINE TRIPHOSPHATE; AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; ELECTROPHYSIOLOGY; GLUTARATES; HIPPOCAMPUS; NERVE DEGENERATION; NERVOUS SYSTEM DISEASES; NEURONS; ORGAN CULTURE TECHNIQUES; RATS;

EID: 8544236222     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/A:1005342331229     Document Type: Conference Paper

References (12)

1. Bennett JP, Logan WJ, Snyder SH (1973) Amino acids as central nervous transmitters: the influence of ions, amino acid analogues, and ontogeny on transport systems for L-glutamic and L-aspartic acids and glycine into central nervous synaptosomes of the rat. J Neurochem 21: 1533-1550.
2. Christensen E (1983) Improved assay of glutaryl-CoA dehydrogenase in cultured cells and liver: application to glutaric aciduria type I. Clin Chim Acta 129: 91-97.
3. Gähwiler BH (1981) Organotypic monolayer cultures of nervous tissue. J Neurosci Methods 4: 329-342.
4. Goodman SI, Norenberg MD, Shikes RH, Breslich DJ, Moe PG (1977) Glutaric aciduria: biochemical and morphological considerations. J Pediatr 90: 746-750.
5. Heyes MP (1987) Hypothesis: a role for quinolinic acid in the neuropathology of glutaric aciduria type I. Can J Neurol Sci 14: 441-443.
6. Land JM, Goulder P, Johnson A, Hockaday J (1992) Glutaric aciduria type I. An atypical presentation together with some observations upon treatment and the possible cause of cerebral damage. Neuropediatrics 23: 322-326.
7. Ludolph AC, Seelig M, Ludolph A, et al (1992) 3-Nitropropionic acid decreases cellular energy levels and causes neuronal degeneration in cortical explants. Neurodegeneration 1: 155-161.
8. Mußhoff U, Madeja M, Bloms-Funke P, Speckmann E-J (1994) Effects of the epileptogenic agent bicuculline methiodide on membrane currents induced by N-methyl-D-aspartate and kainate (oocyte; Xenopus laevis). Brain Res 639: 135-138.
9. Olney JW (1980) Excitotoxic mechanisms of neurotoxicity. In Spencer PS, Schaumburg HH, eds. Clinical and Experimental Neurotoxicology. Williams and Wilkins, Baltimore, 272-294.
10. Stokke O, Goodman SI, Moe PG (1976) Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and β-hydroxyglutarate: explanation of the symptoms in glutaric aciduria? Clin Chim Acta 66: 411-415.
11. Swann JW, Brady RJ, Martin DL (1989) Postnatal development of GABA-mediated synaptic inhibition in rat hippocampus. Neurosci 28: 551-561.
12. Vornov JJ, Tasker RC, Coyle JT (1991) Direct observation of the agonist-specific regional vulnerability to glutamate, NMDA, and kainate neurotoxicity in organotypic hippocampal cultures. Exp Neurol 114: 11-22.