Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.
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Characterization of a monoclonal antibody with specificity for holo-transcobalamin.Striatal neuroprotection with methylene blue.Long-term neurological outcome of a cohort of 80 patients with classical organic acidurias.Profiling of oxidative stress in patients with inborn errors of metabolism.Regional characterization of energy metabolism in the brain of normal and MPTP-intoxicated mice using new markers of glucose and phosphate transport.HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase.Asthma, allergy, and responses to methyl donor supplements and nutrientsMaleic Acid--but Not Structurally Related Methylmalonic Acid--Interrupts Energy Metabolism by Impaired Calcium Homeostasis.Neurocognitive phenotype of isolated methylmalonic acidemiaPopulation prevalence, attributable risk, and attributable risk percentage for high methylmalonic acid concentrations in the post-folic acid fortification period in the US.Brain damage in methylmalonic aciduria: 2-methylcitrate induces cerebral ammonium accumulation and apoptosis in 3D organotypic brain cell cultures.Mitochondrial Factors and VACTERL Association-Related Congenital MalformationsMitochondrial dysfunction in mut methylmalonic acidemia.Neurometabolic mechanisms for memory enhancement and neuroprotection of methylene blue."Classical organic acidurias": diagnosis and pathogenesis.Glutathione as a Redox Biomarker in Mitochondrial Disease-Implications for Therapy.High-Level γ-Glutamyl-Hydrolase (GGH) Expression is Linked to Poor Prognosis in ERG Negative Prostate Cancer.Evidence of Oxidative Stress and Secondary Mitochondrial Dysfunction in Metabolic and Non-Metabolic Disorders.Novel Mouse Models of Methylmalonic Aciduria Recapitulate Phenotypic Traits with a Genetic Dosage Effect.Evidence of redox imbalance in a patient with succinic semialdehyde dehydrogenase deficiencySecondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.Methylmalonic acid, a biochemical hallmark of methylmalonic acidurias but no inhibitor of mitochondrial respiratory chain.Expanding the Spectrum of Methylmalonic Acid-Induced Pallidal Stroke: First Reported Case of Metabolic Globus Pallidus Stroke in Transcobalamin II Deficiency.NMDA receptor activation and respiratory chain complex V inhibition contribute to neurodegeneration in d-2-hydroxyglutaric aciduria.Bioenergetics in glutaryl-coenzyme A dehydrogenase deficiency: a role for glutaryl-coenzyme A.Maternal folic acid use during pregnancy, methylenetetrahydrofolate reductase gene polymorphism, and child's lung function and asthma.Organic acidurias in adults: late complications and management.Disorders of branched chain amino acid metabolism.
P2860
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P2860
Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.
description
2002 nî lūn-bûn
@nan
2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
@zh-sg
2002年學術文章
@yue
2002年學術文章
@zh
2002年學術文章
@zh-hant
name
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@en
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@nl
type
label
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@en
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@nl
prefLabel
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@en
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@nl
P2093
P356
P1476
Neurodegeneration in methylmal ...... tically acting excitotoxicity.
@en
P2093
Angela Hinz
Ertan Mayatepek
Friederike Hörster
Georg F Hoffmann
Jürgen G Okun
Klaus Unsicker
Lilla M Farkas
Patrik Feyh
Stefan Kölker
Sven Sauer
P304
14674-14680
P356
10.1074/JBC.M200997200
P407
P577
2002-02-14T00:00:00Z