Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
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Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegiaExome sequencing identifies mitochondrial alanyl-tRNA synthetase mutations in infantile mitochondrial cardiomyopathyA mitochondrial RNAi screen defines cellular bioenergetic determinants and identifies an adenylate kinase as a key regulator of ATP levelsSyndromic parkinsonism and dementia associated with OPA1 missense mutationsThe mtDNA-encoded ND6 subunit of mitochondrial NADH dehydrogenase is essential for the assembly of the membrane arm and the respiratory function of the enzyme.Upregulation of Mitochondrial Content in Cytochrome c Oxidase Deficient FibroblastsTreatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effectsGlutamyl-tRNAGln amidotransferase is essential for mammalian mitochondrial translation in vivoFunctional Characterization of Three Concomitant MtDNA LHON Mutations Shows No Synergistic Effect on Mitochondrial ActivityNSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNA(Met)Mitochondrial dysfunction remodels one-carbon metabolism in human cellsMutations in MDH2, Encoding a Krebs Cycle Enzyme, Cause Early-Onset Severe EncephalopathyQIL1 mutation causes MICOS disassembly and early onset fatal mitochondrial encephalopathy with liver diseaseProteomic mapping of cytosol-facing outer mitochondrial and ER membranes in living human cells by proximity biotinylationThe glutamate/cystine xCT antiporter antagonizes glutamine metabolism and reduces nutrient flexibility.Galactose enhances oxidative metabolism and reveals mitochondrial dysfunction in human primary muscle cellsImpaired mitochondrial function in human placenta with increased maternal adiposityEffects of inhibiting CoQ10 biosynthesis with 4-nitrobenzoate in human fibroblasts.Membrane-bound CYB5R3 is a common effector of nutritional and oxidative stress response through FOXO3a and Nrf2.Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency.RIP1 maintains DNA integrity and cell proliferation by regulating PGC-1α-mediated mitochondrial oxidative phosphorylation and glycolysisMutations in GTPBP3 cause a mitochondrial translation defect associated with hypertrophic cardiomyopathy, lactic acidosis, and encephalopathyOestrogens ameliorate mitochondrial dysfunction in Leber's hereditary optic neuropathy.Disruptive mitochondrial DNA mutations in complex I subunits are markers of oncocytic phenotype in thyroid tumors.Screen for mitochondrial DNA copy number maintenance genes reveals essential role for ATP synthaseAn MRPS12 mutation modifies aminoglycoside sensitivity caused by 12S rRNA mutations.Identification of fumarate hydratase inhibitors with nutrient-dependent cytotoxicity.Efficient mitochondrial biogenesis drives incomplete penetrance in Leber's hereditary optic neuropathy.LHON: Mitochondrial Mutations and More.Nutrient-sensitized screening for drugs that shift energy metabolism from mitochondrial respiration to glycolysis.Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells.Measurement of Systemic Mitochondrial Function in Advanced Primary Open-Angle Glaucoma and Leber Hereditary Optic Neuropathy.FASTKD2 is an RNA-binding protein required for mitochondrial RNA processing and translation.The Effect of a Novel c.820C>T (Arg274Trp) Mutation in the Mitofusin 2 Gene on Fibroblast Metabolism and Clinical Manifestation in a Patient.Molecular hydrogen protects against oxidative stress-induced SH-SY5Y neuroblastoma cell death through the process of mitohormesis.Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophyGene knockout using transcription activator-like effector nucleases (TALENs) reveals that human NDUFA9 protein is essential for stabilizing the junction between membrane and matrix arms of complex IHuman Cytomegalovirus Infection Upregulates the Mitochondrial Transcription and Translation MachineriesMechanisms of human mitochondrial DNA maintenance: the determining role of primary sequence and length over function.ATG7 regulates energy metabolism, differentiation and survival of Philadelphia-chromosome-positive cells.
P2860
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P2860
Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts.
description
1992 nî lūn-bûn
@nan
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
1992年论文
@zh
1992年论文
@zh-cn
name
Nonviability of cells with oxi ...... affected patient fibroblasts.
@en
type
label
Nonviability of cells with oxi ...... affected patient fibroblasts.
@en
prefLabel
Nonviability of cells with oxi ...... affected patient fibroblasts.
@en
P2093
P1476
Nonviability of cells with oxi ...... affected patient fibroblasts.
@en
P2093
Petrova-Benedict R
Robinson BH
Wallace DC
P304
P356
10.1016/0885-4505(92)90056-5
P577
1992-10-01T00:00:00Z