review article | Q7318358 |
scholarly article | Q13442814 |
P356 | DOI | 10.1016/S0098-2997(02)00013-4 |
P698 | PubMed publication ID | 12231008 |
P2093 | author name string | Vitaliy B Borisov | |
P2860 | cites work | Heme deficiency selectively interrupts assembly of mitochondrial complex IV in human fibroblasts: revelance to aging | Q74614648 |
Fatty acids as natural uncouplers preventing generation of O2.- and H2O2 by mitochondria in the resting state | Q77377987 | ||
Missense mutation in the mtDNA cytochrome b gene in a patient with myopathy | Q77568402 | ||
Heme/Copper Terminal Oxidases | Q77646446 | ||
A nonsense mutation (G15059A) in the cytochrome b gene in a patient with exercise intolerance and myoglobinuria | Q77813538 | ||
Spinal muscular atrophy-like picture, cardiomyopathy, and cytochrome c oxidase deficiency | Q77951839 | ||
SURF1, encoding a factor involved in the biogenesis of cytochrome c oxidase, is mutated in Leigh syndrome | Q22008476 | ||
Identification and characterization of human cDNAs specific to BCS1, PET112, SCO1, COX15, and COX11, five genes involved in the formation and function of the mitochondrial respiratory chain | Q22008622 | ||
Fatal infantile cardioencephalomyopathy with COX deficiency and mutations in SCO2, a COX assembly gene | Q22010737 | ||
A mutation in the human heme A:farnesyltransferase gene (COX10 ) causes cytochrome c oxidase deficiency | Q22253915 | ||
Ubiquinol-cytochrome-c reductase from human and bovine mitochondria | Q24321271 | ||
Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency. Abnormalities of several iron-sulfur proteins | Q24594335 | ||
The whole structure of the 13-subunit oxidized cytochrome c oxidase at 2.8 A | Q27732721 | ||
Crystal structure of the cytochrome bc1 complex from bovine heart mitochondria | Q27739958 | ||
Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex. | Q27931821 | ||
A microdeletion in cytochrome c oxidase (COX) subunit III associated with COX deficiency and recurrent myoglobinuria | Q28115983 | ||
A missense mutation of cytochrome oxidase subunit II causes defective assembly and myopathy | Q28118731 | ||
Expression and functional analysis of SURF1 in Leigh syndrome patients with cytochrome c oxidase deficiency | Q28137735 | ||
Missense mutations in SURF1 associated with deficient cytochrome c oxidase assembly in Leigh syndrome patients | Q28140418 | ||
Mutations in SCO2 are associated with a distinct form of hypertrophic cardiomyopathy and cytochrome c oxidase deficiency | Q28140889 | ||
A mitochondrial cytochrome b mutation but no mutations of nuclearly encoded subunits in ubiquinol cytochrome c reductase (complex III) deficiency | Q28142495 | ||
Mutations in the SURF1 gene associated with Leigh syndrome and cytochrome C oxidase deficiency | Q28214123 | ||
Familial mitochondrial encephalomyopathy (MERRF): genetic, pathophysiological, and biochemical characterization of a mitochondrial DNA disease | Q67950234 | ||
Progressive cytochrome c oxidase deficiency in a case of Leigh's encephalomyelopathy | Q68459547 | ||
Cytochrome c oxidase deficiency | Q68488901 | ||
Mitochondrial myopathies: clinical and biochemical features of 30 patients with major deletions of muscle mitochondrial DNA | Q69414414 | ||
de Toni-Fanconi-Debré syndrome with Leigh syndrome revealing severe muscle cytochrome c oxidase deficiency | Q69824858 | ||
A newborn infant with respiratory distress and persistent stridulous breathing | Q69830696 | ||
Defects in the cytochrome bc1 complex in mitochondrial diseases | Q69834284 | ||
Ubisemiquinone is the electron donor for superoxide formation by complex III of heart mitochondria | Q70080329 | ||
Myopathy and fatal cardiopathy due to cytochrome c oxidase deficiency | Q70152990 | ||
Benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase deficiency | Q70167731 | ||
Decreased cytochrome oxidase activity in hepatic mitochondria after chronic ethanol consumption and the possible role of decreased cytochrome aa3 content and changes in phospholipids | Q70189847 | ||
A novel gly290asp mitochondrial cytochrome b mutation linked to a complex III deficiency in progressive exercise intolerance | Q71763922 | ||
Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria | Q72097479 | ||
Altered kinetics of cytochrome c oxidase in a patient with severe mitochondrial encephalomyopathy | Q72184065 | ||
Immunohistochemical analysis of muscle cytochrome c oxidase deficiency in children | Q72207091 | ||
Early-onset cerebellar ataxia, myoclonus and hypogonadism in a case of mitochondrial complex III deficiency treated with vitamins K3 and C | Q72351955 | ||
Liver cytochrome c oxidase deficiency in a case of neonatal-onset hepatic failure | Q72416019 | ||
Exercise intolerance due to mutations in the cytochrome b gene of mitochondrial DNA | Q73031929 | ||
Biochemical, genetic and immunoblot analyses of 17 patients with an isolated cytochrome c oxidase deficiency | Q73089677 | ||
An out-of-frame cytochrome b gene deletion from a patient with parkinsonism is associated with impaired complex III assembly and an increase in free radical production | Q73192853 | ||
Cytochrome oxidase immunohistochemistry: clues for genetic mechanisms | Q73472189 | ||
Mitochondrial myopathy, parkinsonism, and multiple mtDNA deletions in a Sephardic Jewish family | Q73685069 | ||
A pathogenic 15-base pair deletion in mitochondrial DNA-encoded cytochrome c oxidase subunit III results in the absence of functional cytochrome c oxidase | Q73731474 | ||
Differential features of patients with mutations in two COX assembly genes, SURF-1 and SCO2 | Q73775066 | ||
Pregnancy with cytochrome oxidase-deficient mitochondrial myopathy | Q73835431 | ||
A new mtDNA mutation in the tRNA(Leu(UUR)) gene associated with ocular myopathy | Q74014562 | ||
Combined enzymatic complex I and III deficiency associated with mutations in the nuclear encoded NDUFS4 gene | Q74177562 | ||
A missense mutation in the mitochondrial cytochrome b gene in a revisited case with histiocytoid cardiomyopathy | Q74228370 | ||
Early-onset multisystem mitochondrial disorder caused by a nonsense mutation in the mitochondrial DNA cytochrome C oxidase II gene | Q74518028 | ||
Isolation of a candidate gene for Menkes disease and evidence that it encodes a copper-transporting ATPase | Q28269082 | ||
Neurodegeneration in the animal model of Menkes' disease involves Bcl-2-linked apoptosis | Q28588740 | ||
Cytochrome c oxidase in Alzheimer's disease brain: purification and characterization | Q30465592 | ||
Atypical MELAS syndrome associated with a new mitochondrial tRNA glutamine point mutation | Q31921055 | ||
Cytochrome c oxidase deficient cells accumulate in the hippocampus and choroid plexus with age. | Q31968612 | ||
Organization of multiple nucleoids and DNA molecules in mitochondria of a human cell | Q33265798 | ||
Cytochrome c oxidase deficiency presenting as recurrent neonatal myoglobinuria | Q33488560 | ||
Mitochondrial involvement in Alzheimer's disease | Q33540918 | ||
Structural basis of multifunctional bovine mitochondrial cytochrome bc1 complex | Q33790884 | ||
Mitochondrial physiology and pathology; concepts of programmed death of organelles, cells and organisms | Q33811822 | ||
Mitochondrial respiratory chain diseases and mutations in nuclear DNA: a promising start? | Q33961460 | ||
A mutant mitochondrial respiratory chain assembly protein causes complex III deficiency in patients with tubulopathy, encephalopathy and liver failure. | Q34088669 | ||
Mitochondrial encephalomyopathy and complex III deficiency associated with a stop-codon mutation in the cytochrome b gene | Q34144271 | ||
Effects of mutations in mitochondrial cytochrome b in yeast and man. Deficiency, compensation and disease. | Q34169851 | ||
Mitochondrial DNA mutations in disease and ageing. | Q34203016 | ||
Ubiquinone (coenzyme q10) and mitochondria in oxidative stress of parkinson's disease | Q34249987 | ||
Mitochondrial function and alzheimer's disease | Q34249993 | ||
Cytochrome c oxidase deficiency associated with the first stop-codon point mutation in human mtDNA. | Q34386122 | ||
A high rate (20%-30%) of parental consanguinity in cytochrome-oxidase deficiency. | Q34386504 | ||
Mutations of SURF-1 in Leigh disease associated with cytochrome c oxidase deficiency | Q34388122 | ||
A stop-codon mutation in the human mtDNA cytochrome c oxidase I gene disrupts the functional structure of complex IV. | Q34390115 | ||
A novel frameshift mutation of the mtDNA COIII gene leads to impaired assembly of cytochrome c oxidase in a patient affected by Leigh-like syndrome | Q34511770 | ||
Structure at 2.8 Å resolution of cytochrome c oxidase from Paracoccus denitrificans | Q34662350 | ||
Oxygen activation and the conservation of energy in cell respiration | Q35226038 | ||
Structure and function of cytochrome c oxidase | Q37886474 | ||
Vectorial electron and proton transfer steps in the cytochrome bc1 complex | Q37891006 | ||
Cytochrome-c oxidase. Subunit structure and proton pumping. | Q38181156 | ||
Cytochrome c oxidase-deficient patients have distinct subunit assembly profiles | Q40816638 | ||
Lacticacidemia | Q40859208 | ||
A single cell complementation class is common to several cases of cytochrome c oxidase-defective Leigh's syndrome. | Q41129950 | ||
Evidence for physiological down-regulation of brain oxidative phosphorylation in Alzheimer's disease | Q41206150 | ||
Subunit specific monoclonal antibodies show different steady-state levels of various cytochrome-c oxidase subunits in chronic progressive external ophthalmoplegia | Q41206220 | ||
Human cytochrome c oxidase: structure, function, and deficiency. | Q41547376 | ||
Evidence for a mitochondrial oxidative phosphorylation defect in brains from patients with schizophrenia | Q42501952 | ||
Deafferentation of the septo-hippocampal pathway in rats as a model of the metabolic events in Alzheimer's disease | Q42503868 | ||
Leigh disease: clinical, neuroradiologic, and biochemical study of three new cases with cytochrome c oxidase deficiency | Q42508837 | ||
Adefovir nephrotoxicity: possible role of mitochondrial DNA depletion | Q43526884 | ||
New splicing-site mutations in the SURF1 gene in Leigh syndrome patients | Q43560233 | ||
Dramatic improvement in mitochondrial cardiomyopathy following treatment with idebenone | Q43564490 | ||
Apoptosis and ROS detoxification enzymes correlate with cytochrome c oxidase deficiency in mitochondrial encephalomyopathies. | Q43581937 | ||
The mitochondrial DNA mutation T12297C affects a highly conserved nucleotide of tRNA(Leu(CUN)) and is associated with dilated cardiomyopathy | Q43583234 | ||
Abnormal calcium homeostasis in fibroblasts from patients with Leigh disease. | Q43600254 | ||
Ethanol decreases the expression of mitochondrial cytochrome c oxidase mRNA in the rat. | Q43619951 | ||
Energy hypometabolism in posterior cingulate cortex of Alzheimer's patients: superficial laminar cytochrome oxidase associated with disease duration. | Q43653444 | ||
A novel nonsense mutation (Q352X) in the mitochondrial cytochrome b gene associated with a combined deficiency of complexes I and III. | Q43814295 | ||
Myoclonic epilepsy and ragged-red fibers with cytochrome oxidase deficiency: neuropathology, biochemistry, and molecular genetics | Q44023731 | ||
Identification of the electron transfers in cytochrome oxidase that are coupled to proton-pumping | Q44352752 | ||
Increased sensitivity of cerebrohepatorenal syndrome fibroblasts to antimycin A | Q44493978 | ||
Cytochrome b mutations in Leber hereditary optic neuropathy | Q44775364 | ||
Cytochrome c oxidase: structure and spectroscopy | Q47910949 | ||
Cytochrome c oxidase as a proton-pumping peroxidase: reaction cycle and electrogenic mechanism | Q47983275 | ||
Crystal structure of bovine heart cytochrome c oxidase at 2.8 A resolution | Q47983409 | ||
Molecular analysis of cytochrome c oxidase deficiency in Leigh's syndrome | Q48054632 | ||
A new mutation associated with MELAS is located in a mitochondrial DNA polypeptide-coding gene | Q48071183 | ||
Electron transport chain defects in Alzheimer's disease brain | Q48107929 | ||
Clinical and biochemical studies on cytochrome oxidase deficiencies | Q48162574 | ||
Cytochrome c oxidase deficiency in Leigh syndrome | Q48203324 | ||
MELAS: clinical features, biochemistry, and molecular genetics | Q48496312 | ||
Cytochrome c oxidase subunit I microdeletion in a patient with motor neuron disease. | Q48550765 | ||
Cytochrome C oxidase-deficient mitochondria in mitochondrial myopathy | Q48601404 | ||
Mitochondrial cytochrome deficiency presenting as a myopathy with hypotonia, external ophthalmoplegia, and lactic acidosis in an infant and as fatal hepatopathy in a second cousin. | Q48735604 | ||
A SURF1 gene mutation presenting as isolated leukodystrophy | Q48839472 | ||
Progressive poliodystrophy (Alpers' disease) with a defect in cytochrome aa3 in muscle: a report of two unrelated patients | Q48846738 | ||
Diagnosis of cyanide intoxication by measurement of cytochrome c oxidase activity | Q48909501 | ||
A case of MERRF associated with chronic pancreatitis | Q48921665 | ||
Vascular involvement in benign infantile mitochondrial myopathy caused by reversible cytochrome c oxidase deficiency | Q48971246 | ||
Two point mutations in mitochondrial DNA of cytochrome c oxidase coexist with normal mtDNA in a patient with Alzheimer's disease | Q48973485 | ||
Leigh syndrome: clinical features and biochemical and DNA abnormalities. | Q49107059 | ||
SURFEIT-1 gene analysis and two-dimensional blue native gel electrophoresis in cytochrome c oxidase deficiency. | Q50515850 | ||
Characterization of SURF-1 expression and Surf-1p function in normal and disease conditions. | Q50515893 | ||
Focal cytochrome c oxidase deficiency in the brain and dorsal root ganglia in a case with mitochondrial encephalomyopathy (tRNAIle 4269 mutation): histochemical, immunohistochemical, and ultrastructural study | Q50518910 | ||
Lactic Acidosis and Mitochondrial Myopathy Associated with Deficiency of Several Components of Complex III of the Respiratory Chain | Q51641269 | ||
Reduced platelet cytochrome c oxidase activity in Alzheimer's disease. | Q53207411 | ||
Functional alterations in Alzheimer's disease: selective loss of mitochondrial-encoded cytochrome oxidase mRNA in the hippocampal formation. | Q53316227 | ||
Two novel mutations of SURF1 in Leigh syndrome with cytochrome c oxidase deficiency | Q56591758 | ||
A Novel Mutation in SURF1 Causes Skipping of Exon 8 in a Patient with Cytochrome c Oxidase-Deficient Leigh Syndrome and Hypertrichosis | Q56866594 | ||
Loss-of-function mutations of SURF-1 are specifically associated with Leigh syndrome with cytochromec oxidase deficiency | Q57251902 | ||
Age-related mitochondrial genotypic and phenotypic alterations in human skeletal muscle | Q60532160 | ||
Maternally inherited myopathy and cardiomyopathy: association with mutation in mitochondrial DNA tRNALeu(UUR) | Q61606262 | ||
Antimycin resistance and ubiquinol cytochrome c reductase instability associated with a human cytochrome b mutation. | Q64897786 | ||
Clinical heterogeneity in respiratory chain complex III deficiency in childhood | Q67208792 | ||
P433 | issue | 5 | |
P407 | language of work or name | English | Q1860 |
P304 | page(s) | 385-412 | |
P577 | publication date | 2002-10-01 | |
P1433 | published in | Molecular Aspects of Medicine | Q6895933 |
P1476 | title | Defects in mitochondrial respiratory complexes III and IV, and human pathologies. | |
P478 | volume | 23 |
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Q35203821 | High-level inhibition of mitochondrial complexes III and IV is required to increase glutamate release from the nerve terminal |
Q36727229 | Isolated cytochrome c oxidase deficiency in G93A SOD1 mice overexpressing CCS protein |
Q39016389 | Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment. |
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