scholarly article | Q13442814 |
P356 | DOI | 10.1007/S10545-012-9511-0 |
P698 | PubMed publication ID | 22767283 |
P50 | author | Andrey Y. Abramov | Q48291443 |
P2093 | author name string | Shamima Rahman | |
Iain P Hargreaves | |||
Simon J Heales | |||
Kate E C Duberley | |||
Annapurna Chalasani | |||
P2860 | cites work | Protein measurement with the Folin phenol reagent | Q20900776 |
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye binding | Q25938984 | ||
Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis | Q28115898 | ||
Multiple neurotransmitter synthesis by human neuroblastoma cell lines and clones | Q28285883 | ||
Blue native PAGE | Q28296330 | ||
Muscle coenzyme Q deficiency in familial mitochondrial encephalomyopathy | Q28360128 | ||
Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations | Q33742421 | ||
Mitochondrial defects in cardiomyopathy and neuromuscular disease | Q33825717 | ||
Coenzyme Q deficiency triggers mitochondria degradation by mitophagy | Q34015447 | ||
Metabolism and function of coenzyme Q. | Q34294224 | ||
Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency | Q34378808 | ||
Coenzyme Q and the regulation of intracellular steady-state levels of superoxide in HL-60 cells | Q34551992 | ||
176th ENMC International Workshop: diagnosis and treatment of coenzyme Q₁₀ deficiency | Q35569154 | ||
Clinical, biochemical and molecular aspects of cerebellar ataxia and Coenzyme Q10 deficiency. | Q36825734 | ||
Poly-ADP ribose polymerase activates nuclear proteasome to degrade oxidatively damaged histones | Q37202186 | ||
Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency | Q37317328 | ||
PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death | Q39873016 | ||
Functional assays in high-resolution clear native gels to quantify mitochondrial complexes in human biopsies and cell lines | Q40062365 | ||
Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption | Q40067062 | ||
Cell metabolism affects selective vulnerability in PINK1-associated Parkinson's disease | Q40394013 | ||
Reactive oxygen species, mitochondria, apoptosis and aging. | Q41601406 | ||
Uncoupling: new approaches to an old problem of bioenergetics | Q41723090 | ||
Generation of superoxide anion by the NADH dehydrogenase of bovine heart mitochondria | Q41829151 | ||
Cytochrome c oxidase and cardiolipin alterations in response to skeletal muscle ischaemia and reperfusion | Q41876549 | ||
Role of Nrf2 signaling in regulation of antioxidants and phase 2 enzymes in cardiac fibroblasts: protection against reactive oxygen and nitrogen species-induced cell injury | Q42478374 | ||
Sequential damage in mitochondrial complexes by peroxidative stress | Q44438376 | ||
Progression despite replacement of a myopathic form of coenzyme Q10 defect | Q45028156 | ||
The oxidative inactivation of mitochondrial electron transport chain components and ATPase. | Q46025170 | ||
4-Hydroxybenzoate:Polyprenyl transferase and the prenylation of 4-aminobenzoate in mammalian tissues | Q48433423 | ||
Energy thresholds in brain mitochondria. Potential involvement in neurodegeneration. | Q48462986 | ||
Depletion of brain glutathione is accompanied by impaired mitochondrial function and decreased N-acetyl aspartate concentration | Q49204906 | ||
Determination of coenzyme Q10 status in blood mononuclear cells, skeletal muscle, and plasma by HPLC with di-propoxy-coenzyme Q10 as an internal standard. | Q53647703 | ||
Coenzyme Q10-responsive ataxia: 2-year-treatment follow-up | Q58184277 | ||
Neonatal presentation of coenzyme Q10 deficiency | Q59463049 | ||
Transition Metals, Ferritin, Glutathione, and Ascorbic Acid in Parkinsonian Brains | Q61714001 | ||
Mechanism of O2- generation in reduction and oxidation cycle of ubiquinones in a model of mitochondrial electron transport systems | Q69844120 | ||
Ubisemiquinone is the electron donor for superoxide formation by complex III of heart mitochondria | Q70080329 | ||
Mitochondrial respiratory chain defects are not accompanied by an increase in the activities of lactate dehydrogenase or manganese superoxide dismutase in paediatric skeletal muscle biopsies | Q73287564 | ||
Mitochondrial damage induced by conditions of oxidative stress | Q77814514 | ||
Glutathione and glutathione-linked enzymes in normal human aortic smooth muscle cells: chemical inducibility and protection against reactive oxygen and nitrogen species-induced injury | Q79501744 | ||
P433 | issue | 1 | |
P921 | main subject | pathogenesis | Q372016 |
mitochondrion | Q39572 | ||
4-aminobenzoic acid | Q284959 | ||
ubidecarenone | Q321285 | ||
P304 | page(s) | 63-73 | |
P577 | publication date | 2012-07-06 | |
P1433 | published in | Journal of Inherited Metabolic Disease | Q6295359 |
P1476 | title | Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment | |
P478 | volume | 36 |
Q41827616 | 'Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich's ataxia'. |
Q36443232 | Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes |
Q41456657 | Coq6 is responsible for the C4-deamination reaction in coenzyme Q biosynthesis in Saccharomyces cerevisiae |
Q99549449 | Disorders of Human Coenzyme Q10 Metabolism: An Overview |
Q51538858 | Drug-Induced Mitochondrial Toxicity. |
Q36061158 | Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice. |
Q39424489 | Impact of Chemical Analogs of 4-Hydroxybenzoic Acid on Coenzyme Q Biosynthesis: From Inhibition to Bypass of Coenzyme Q Deficiency. |
Q89664128 | L-type Ca2+ channel-mediated Ca2+ influx adjusts neuronal mitochondrial function to physiological and pathophysiological conditions |
Q41674714 | Measurement of Respiratory Chain Enzyme Activity in Human Renal Biopsy Specimens |
Q48103576 | Mitochondrial CoQ deficiency is a common driver of mitochondrial oxidants and insulin resistance. |
Q50999542 | Neutrophil depletion improves diet-induced non-alcoholic fatty liver disease in mice. |
Q40122413 | New susceptible variant of COQ2 gene in Japanese patients with sporadic multiple system atrophy |
Q33687447 | Pathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates. |
Q33956929 | Pathomechanisms in coenzyme q10-deficient human fibroblasts |
Q64079251 | Rapamycin administration is not a valid therapeutic strategy for every case of mitochondrial disease |
Q38690806 | Redox Signaling Mediated by Thioredoxin and Glutathione Systems in the Central Nervous System. |
Q28607038 | The WOMED model of benign thyroid disease: Acquired magnesium deficiency due to physical and psychological stressors relates to dysfunction of oxidative phosphorylation |
Q36853449 | The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome |
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