Glutaric aciduria type I: Pathomechanisms of neurodegeneration
Data up to Jan 2025
Total Citations Per Year
Abstract
References (40)
The Metabolic and Molecular Bases of Inherited Disease
1995 • 12,086 citations
Developmental and regional expression in the rat brain and functional properties of four NMDA receptors
1994 • 3,437 citations
A simple method for organotypic cultures of nervous tissue
1991 • 3,075 citations
Distribution of N-methyl-D-aspartate-sensitive L-[3H]glutamate-binding sites in rat brain
1985 • 1,294 citations
Quantitative determination of glutamate mediated cortical neuronal injury in cell culture by lactate dehydrogenase efflux assay
1987 • 1,280 citations
The release and uptake of excitatory amino acids
1990 • 1,090 citations
Organotypic monolayer cultures of nervous tissue
1981 • 862 citations
Cytotoxic effects of acidic and sulphur containing amino acids on the infant mouse central nervous system
1971 • 808 citations
Protection of substantia nigra from MPP+ neurotoxicity by N-methyl-D-aspartate antagonists
1991 • 554 citations
Oligodendrocytes from forebrain are highly vulnerable to AMPA/kainate receptor-mediated excitotoxicity
1998 • 538 citations
NMDA Receptor Heterogeneity During Postnatal Development of the Rat Brain: Differential Expression of the NR2A, NR2B, and NR2C Subunit Proteins
1997 • 439 citations
Continuous monitoring of ATP-converting reactions by purified firefly luciferase
1976 • 318 citations
AMINO ACIDS AS CENTRAL NERVOUS TRANSMITTERS: THE INFLUENCE OF IONS, AMINO ACID ANALOGUES, AND ONTOGENY ON TRANSPORT SYSTEMS for <scp>l</scp>‐GLUTAMIC AND <scp>l</scp>‐ASPARTIC ACIDS AND GLYCINE INTO CENTRAL NERVOUS SYNAPTOSOMES OF THE RAT1
1973 • 261 citations
Development of the hippocampus in vitro: Cell types, synapses and receptors
1984 • 210 citations
Glutaric aciduria: Biochemical and morphologic considerations
1977 • 196 citations
Direct observation of the agonist-specific regional vulnerability to glutamate, NMDA, and kainate neurotoxicity in organotypic hippocampal cultures
1991 • 175 citations
Mechanisms underlying initiation of excitotoxicity associated with metabolic inhibition.
1991 • 157 citations
Neurotoxicity at the N‐Methyl‐D‐Aspartate Receptor in Energy‐Compromised Neurons An Hypothesis for Cell Death in Aging and Disease
1989 • 157 citations
The regional vulnerability to hypoglycemia-induced neurotoxicity in organotypic hippocampal culture: protection by early tetrodotoxin or delayed MK-801
1992 • 140 citations
Evidence that the Loss of the Voltage‐Dependent Mg2+ Block at the N‐Methyl‐D‐Aspartate Receptor Underlies Receptor Activation During Inhibition of Neuronal Metabolism
1992 • 137 citations
Topical Review: Glutamate in Neurologic Diseases
1997 • 129 citations
Glutaric acidemia
1980 • 113 citations
Formation of layer-specific fiber projections to the hippocampus in vitro.
1993 • 109 citations
Inhibition of brain glutamate decarboxylase by glutarate, glutaconate, and β-Hydroxyglutarate: Explanation of the symptoms in glutaric aciduria?
1976 • 92 citations
Glutamate and γ‐Aminobutyric Acid Neurotransmitter Systems in the Acute Phase of Maple Syrup Urine Disease and Citrullinemia Encephalopathies in Newborn Calves
1992 • 77 citations
Hypothesis: A Role for Quinolinic Acid in the Neuropathology of Glutaric Aciduria Type I
1987 • 69 citations
Improved assay of glutaryl-CoA dehydrogenase in cultured cells and liver: application to glutaric aciduria type I
1983 • 61 citations
Nerve cell lesions caused by 3‐hydroxyglutaric acid: A possible mechanism for neurodegeneration in glutaric acidaemia I
1997 • 59 citations
Striatal degeneration and spongy myelinopathy in glutaric acidemia
1992 • 55 citations
Role of excitotoxins in developmental neuropathology.
1993 • 46 citations
Homocysteic and homocysteine sulphinic acid exhibit excitotoxicity in organotypic cultures from rat brain
1998 • 39 citations
Glutaric Aciduria Type 1 - An Atypical Presentation Together with some Observations upon Treatment and the Possible Cause of Cerebral Damage
1992 • 37 citations
Ketotic Episodes in Glutaryl-CoA Dehydrogenase Deficiency (Glutaric Aciduria)
1979 • 32 citations
Some chemical highlights in the development of excitatory amino acid pharmacology
1991 • 31 citations
The inhibition of mouse brain glutamate decarboxylase by some structural analogues of L-glutamic acid
1977 • 31 citations
Prenatal Diagnosis of Inherited Metabolic Disorders by Stable Isotope Dilution GC‐MS Analysis of Metabolites in Amniotic Fluid: Review of Four Years Experience
1989 • 25 citations
Regulation of Mitochondrial ATP Synthase Activity in Human Myocardium
1998 • 22 citations
Effects of the epileptogenic agent bicuculline methiodide on membrane currents induced byN-methyl-d-aspartate and kainate (oocyte;Xenopus laevis)
1994 • 12 citations
NMDA receptor levels in chronically depolarized long‐term neonatal rat neocortical explants
1992 • 9 citations
Deficiency of pyrimidine 5′‐nucleotidase in human leukocytes
1988 • 8 citations