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Energy and Potassium Ion Homeostasis during Gamma OscillationsMitochondrial disorders in children: toward development of small-molecule treatment strategiesMitochondrial geneticsAlzheimer's pathogenesis and its link to the mitochondrionLoss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in miceDynamics of Human Mitochondrial Complex I Assembly: Implications for Neurodegenerative DiseasesCell Death and Heart Failure in Obesity: Role of Uncoupling ProteinsPreventing Alzheimer's disease by means of natural selectionEnhanced neuronal glucose transporter expression reveals metabolic choice in a HD Drosophila modelOxygen consumption deficit in Huntington disease mouse brain under metabolic stressSulfite Oxidase Activity of Cytochrome c: Role of Hydrogen PeroxideMinnelide/Triptolide Impairs Mitochondrial Function by Regulating SIRT3 in P53-Dependent Manner in Non-Small Cell Lung CancerTMEM65 is a mitochondrial inner-membrane protein.A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.The neuroimaging of Leigh syndrome: case series and review of the literature.Clinical spectrum of females with HCCS mutation: from no clinical signs to a neonatal lethal form of the microphthalmia with linear skin defects (MLS) syndrome.Control of mitochondrial integrity in ageing and diseaseRegulation of pyruvate metabolism and human diseaseMitochondrial complex I deficiency enhances skeletal myogenesis but impairs insulin signaling through SIRT1 inactivation.The molecular evolution of the Qo motifMitochondrial fusion but not fission regulates larval growth and synaptic development through steroid hormone production.AK2 deficiency compromises the mitochondrial energy metabolism required for differentiation of human neutrophil and lymphoid lineagesHuman thioredoxin 2 deficiency impairs mitochondrial redox homeostasis and causes early-onset neurodegeneration.Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in the Ndufs4fky/fky mouse.CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease.Early gene expression changes in spinal cord from SOD1(G93A) Amyotrophic Lateral Sclerosis animal model.Transformations of the macromolecular landscape at mitochondria during DNA-damage-induced apoptotic cell death.ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy.Biochemical investigation of a human pathogenic mutation in the nuclear ATP5E gene using yeast as a model.Homeostasis of redox status derived from glucose metabolic pathway could be the key to understanding the Warburg effect.Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies.A Role for the Mitochondrial Protein Mrpl44 in Maintaining OXPHOS CapacityRedox signalling and mitochondrial stress responses; lessons from inborn errors of metabolism.Interactions between mitochondrial reactive oxygen species and cellular glucose metabolism.Complex I dysfunction underlies the glycolytic switch in pulmonary hypertensive smooth muscle cells.Multiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy.FASTKD2 is an RNA-binding protein required for mitochondrial RNA processing and translation.Proteomic Data in Morphine Addiction Versus Real Protein Activity: Metabolic Enzymes.Increased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia.Elongator-dependent modification of cytoplasmic tRNALysUUU is required for mitochondrial function under stress conditions.
P2860
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P2860
description
2012 nî lūn-bûn
@nan
2012年の論文
@ja
2012年論文
@yue
2012年論文
@zh-hant
2012年論文
@zh-hk
2012年論文
@zh-mo
2012年論文
@zh-tw
2012年论文
@wuu
2012年论文
@zh
2012年论文
@zh-cn
name
OXPHOS mutations and neurodegeneration
@ast
OXPHOS mutations and neurodegeneration
@en
type
label
OXPHOS mutations and neurodegeneration
@ast
OXPHOS mutations and neurodegeneration
@en
prefLabel
OXPHOS mutations and neurodegeneration
@ast
OXPHOS mutations and neurodegeneration
@en
P2093
P2860
P356
P1433
P1476
OXPHOS mutations and neurodegeneration
@en
P2093
Felix Distelmaier
Jan A M Smeitink
Werner J H Koopman
P2860
P356
10.1038/EMBOJ.2012.300
P407
P577
2012-11-13T00:00:00Z