相关参考文献
注意：仅列出部分参考文献，下载原文获取全部文献信息。Uncoupling, metabolic inhibition and induction of mitochondrial permeability transition in rat liver mitochondria caused by the major long-chain hydroxyl monocarboxylic fatty acids accumulating in LCHAD deficiency
Fernanda Hermes Hickmann et al.
BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS (2015)
Dysfunctional TCA-Cycle Metabolism in Glutamate Dehydrogenase Deficient Astrocytes
Jakob D. Nissen et al.
GLIA (2015)
Cyclosporine A normalizes mitochondrial coupling, reactive oxygen species production, and inflammation and partially restores skeletal muscle maximal oxidative capacity in experimental aortic cross-clamping
Julien Pottecher et al.
JOURNAL OF VASCULAR SURGERY (2013)
Astrocytic energetics during excitatory neurotransmission: What are contributions of glutamate oxidation and glycolysis?
Gerald A. Dienel
NEUROCHEMISTRY INTERNATIONAL (2013)
Brain damage in methylmalonic aciduria: 2-methylcitrate induces cerebral ammonium accumulation and apoptosis in 3D organotypic brain cell cultures
Paris Jafari et al.
ORPHANET JOURNAL OF RARE DISEASES (2013)
Glutamate Pays Its Own Way in Astrocytes
Mary C. McKenna
Frontiers in Endocrinology (2013)
Calcium-induced Cardiac Mitochondrial Dysfunction Is Predominantly Mediated by Cyclosporine A-dependent Mitochondrial Permeability Transition Pore
Chontida Yarana et al.
ARCHIVES OF MEDICAL RESEARCH (2012)
Deletion of glutamate dehydrogenase 1 (Glud1) in the central nervous system affects glutamate handling without altering synaptic transmission
Francesca Frigerio et al.
JOURNAL OF NEUROCHEMISTRY (2012)
Methylmalonate impairs mitochondrial respiration supported by NADH-linked substrates: Involvement of mitochondrial glutamate metabolism
Daniela R. Melo et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2012)
siRNA knock down of glutamate dehydrogenase in astrocytes affects glutamate metabolism leading to extensive accumulation of the neuroactive amino acids glutamate and aspartate
Dorte M. Skytt et al.
NEUROCHEMISTRY INTERNATIONAL (2012)
Neurocognitive Phenotype of Isolated Methylmalonic Acidemia
Colin J. O'Shea et al.
PEDIATRICS (2012)
Mechanism for the uncoupling of oxidative phosphorylation by juliprosopine on rat brain mitochondria
Marcos A. Maioli et al.
TOXICON (2012)
Glutamate dehydrogenase in brain mitochondria: Do lipid modifications and transient metabolon formation influence enzyme activity?
Mary C. McKenna
NEUROCHEMISTRY INTERNATIONAL (2011)
Glutaric aciduria type I and methylmalonic aciduria: Simulation of cerebral import and export of accumulating neurotoxic dicarboxylic acids in in vitro models of the blood-brain barrier and the choroid plexus
Sven W. Sauer et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2010)
Reduction of lipid and protein damage in patients with disorders of propionate metabolism under treatment: a possible protective role of L-carnitine supplementation
Graziela S. Ribas et al.
INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE (2010)
Prevention by L-carnitine of DNA damage induced by propionic and L-methylmalonic acids in human peripheral leukocytes in vitro
Graziela S. Ribas et al.
MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS (2010)
Inhibitory Effects of Adenine Nucleotides on Brain Mitochondrial Permeability Transition
Angela Saito et al.
NEUROCHEMICAL RESEARCH (2010)
Mitochondrial dysfunction in mut methylmalonic acidemia
Randy J. Chandler et al.
FASEB JOURNAL (2009)
Genetic and Cellular Studies of Oxidative Stress in Methylmalonic Aciduria (MMA) Cobalamin Deficiency Type C (cblC) With Homocystinuria (MMACHC)
Eva Richard et al.
HUMAN MUTATION (2009)
Prediction of outcome in isolated methylmalonic acidurias: combined use of clinical and biochemical parameters
F. Hoerster et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2009)
EVIDENCE FOR CATABOLIC PATHWAY OF PROPIONATE METABOLISM IN CNS: EXPRESSION PATTERN OF METHYLMALONYL-CoA MUTASE AND PROPIONYL-CoA CARBOXYLASE ALPHA-SUBUNIT IN DEVELOPING AND ADULT RAT BRAIN
D. Ballhausen et al.
NEUROSCIENCE (2009)
Multiple OXPHOS Deficiency in the Liver, Kidney, Heart, and Skeletal Muscle of Patients With Methylmalonic Aciduria and Propionic Aciduria
Yves de Keyzer et al.
PEDIATRIC RESEARCH (2009)
Methylmalonate inhibits succinate-supported oxygen consumption by interfering with mitochondrial succinate uptake
S. R. Mirandola et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2008)
Looking beyond the basal ganglia:: The spectrum of MRI changes in methylmalonic acidaemia
I. Harting et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2008)
Methylmalonic acidaemia leads to increased production of reactive oxygen, species and induction of apoptosis through the mitochondrial/caspase pathway
E. Richard et al.
JOURNAL OF PATHOLOGY (2007)
3-Hydroxyglutaric acid is transported via the sodium-dependent dicarboxylate transporter NaDC3
Franziska Stellmer et al.
JOURNAL OF MOLECULAR MEDICINE-JMM (2007)
The role of nitric oxide on the convulsive behavior and oxidative stress induced by methylmalonate: An electroencephalographic and neurochemical study
Luiz Fernando Freire Royes et al.
EPILEPSY RESEARCH (2007)
Methylene blue prevents methylmalonate-induced seizures and oxidative damage in rat striatum
Ana Flavia Furian et al.
NEUROCHEMISTRY INTERNATIONAL (2007)
The aetiology of neurological complications of organic acidaemias -: A role for the blood-brain barrier
S. Koelker et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2006)
Propionic acid induces convulsions and protein carbonylation in rats
Flavia Karine Rigo et al.
NEUROSCIENCE LETTERS (2006)
Liver transplantation is not curative for methylmalonic acidopathy caused by methylmalonyl-CoA mutase deficiency
Paige Kaplan et al.
MOLECULAR GENETICS AND METABOLISM (2006)
Quantitative analysis of mitochondrial protein expression in methylmalonic acidemia by two-dimensional difference gel electrophoresis
Eva Richard et al.
JOURNAL OF PROTEOME RESEARCH (2006)
Methylmalonic and propionic aciduria
F Deodato et al.
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS (2006)
Intracerebral accumulation of glutaric and 3-hydroxyglutaric acids secondary to limited flux across the blood-brain barrier constitute a biochemical risk factor for neurodegeneration in glutaryl-CoA dehydrogenase deficiency
SW Sauer et al.
JOURNAL OF NEUROCHEMISTRY (2006)
Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues
LF Pettenuzzo et al.
INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE (2006)
α-Tocopherol protects against pentylenetetrazol- and methylmalonate-induced convulsions
MCP Ribeiro et al.
EPILEPSY RESEARCH (2005)
Properties of the permeability transition pore in mitochondria devoid of cyclophilin D
E Basso et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Involvement of NO in the convulsive behavior and oxidative damage induced by the intrastriatal injection of methylmalonate
LFF Royes et al.
NEUROSCIENCE LETTERS (2005)
Methylmalonic and propionic acidaemias: Management and outcome
HO de Baulny et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2005)
Mitochondrial permeability transition in neuronal damage promoted by Ca2+ and respiratory chain complex II inhibition
EN Maciel et al.
JOURNAL OF NEUROCHEMISTRY (2004)
Intrastriatal methylmalonic acid administration induces convulsions and TBARS production, and alters Na+,K+-ATPase activity in the rat striatum and cerebral cortex
CRM Malfatti et al.
EPILEPSIA (2003)
GM1 ganglioside attenuates convulsions and thiobarbituric acid reactive substances production induced by the intrastriatal injection of methylmalonic acid
MR Fighera et al.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY (2003)
Creatine protects against the convulsive behavior and lactate production elicited by the intrastriatal injection of methylmalonate
LFF Royes et al.
NEUROSCIENCE (2003)
Ascorbic acid prevents cognitive deficits caused by chronic administration of propionic acid to rats in the water maze
LF Pettenuzzo et al.
PHARMACOLOGY BIOCHEMISTRY AND BEHAVIOR (2002)
Inhibition of the mitochondrial respiratory chain complex activities in rat cerebral cortex by methylmalonic acid
AM Brusque et al.
NEUROCHEMISTRY INTERNATIONAL (2002)
Regulation of human glutamate dehydrogenases: Implications for glutamate, ammonia and energy metabolism in brain
A Plaitakis et al.
JOURNAL OF NEUROSCIENCE RESEARCH (2001)
Effects of methylmalonic and propionic acids on glutamate uptake by synaptosomes and synaptic vesicles and on glutamate release by synaptosomes from cerebral cortex of rats
AM Brusque et al.
BRAIN RESEARCH (2001)
Methylmalonic acid induces excitotoxic neuronal damage in vitro
S Kölker et al.
JOURNAL OF INHERITED METABOLIC DISEASE (2000)
Propionic and L-methylmalonic acids induce oxidative stress in brain of young rats
FU Fontella et al.
NEUROREPORT (2000)
分享论文
