Published in J Clin Invest on February 01, 1987
Deficiency of skeletal muscle succinate dehydrogenase and aconitase. Pathophysiology of exercise in a novel human muscle oxidative defect. J Clin Invest (1991) 1.26
Mitochondrial myopathies. J Inherit Metab Dis (1987) 1.06
Nuclear DNA origin of mitochondrial complex I deficiency in fatal infantile lactic acidosis evidenced by transnuclear complementation of cultured fibroblasts. J Clin Invest (1999) 1.04
Impaired mitochondrial beta-oxidation in a patient with an abnormality of the respiratory chain. Studies in skeletal muscle mitochondria. J Clin Invest (1990) 0.97
Congenital deficiency of a 20-kDa subunit of mitochondrial complex I in fibroblasts. Am J Hum Genet (1991) 0.76
Cloning of the human NADH: ubiquinone oxidoreductase subunit B13: localization to chromosome 7q32 and identification of a pseudogene on 11p15. Mamm Genome (1997) 0.76
Cleavage of structural proteins during the assembly of the head of bacteriophage T4. Nature (1970) 1528.65
A case of severe hypermetabolism of nonthyroid origin with a defect in the maintenance of mitochondrial respiratory control: a correlated clinical, biochemical, and morphological study. J Clin Invest (1962) 4.06
Benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase deficiency. Ann Neurol (1983) 1.82
Mitochondrial encephalomyopathies: biochemical studies in two cases revealing defects in the respiratory chain. Brain (1982) 1.80
A mitochondrial myopathy characterized by a deficiency in reducible cytochrome b. Brain (1977) 1.69
Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis. J Clin Invest (1984) 1.42
Studies of factors involved in oxidative phosphorylation. Proc Natl Acad Sci U S A (1962) 1.37
31P NMR examination of two patients with NADH-CoQ reductase deficiency. Nature (1982) 1.37
Fatal infantile mitochondrial myopathy and renal dysfunction due to cytochrome-c-oxidase deficiency. Neurology (1980) 1.25
A mitochondrial myopathy with a deficiency of respiratory chain NADH-CoQ reductase activity. J Neurol Sci (1979) 1.21
The organization of NADH dehydrogenase polypeptides in the inner mitochondrial membrane. Biochem J (1980) 1.10
Deficiency in ubiquinone cytochrome c reductase in a patient with mitochondrial myopathy and lactic acidosis. Proc Natl Acad Sci U S A (1983) 1.06
Neuromuscular disorder associated with a defect in mitochondrial energy supply. Arch Neurol (1976) 1.05
Cytochrome-c-oxidase deficiency in a floppy infant. Neurology (1982) 0.97
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. Ann Neurol (1983) 0.96
Mitochondrial encephalomyopathy with decreased succinate-cytochrome c reductase activity. Neurology (1984) 0.94
Investigation of the iron-sulfur clusters in some mitochondrial mutants of Saccharomyces cerevisiae. A possible correlation between Rieske's iron-sulfur cluster and cytochrome b. Eur J Biochem (1982) 0.93
Mitochondrial myopathy. Biochemical studies revealing a deficiency of NADH--cytochrome b reductase activity. J Neurol Sci (1981) 0.92
Immunological assays of the NADH dehydrogenase content of bovine heart mitochondria and submitochondrial particles. FEBS Lett (1980) 0.86
Sedative but not anxiolytic properties of benzodiazepines are mediated by the GABA(A) receptor alpha1 subtype. Nat Neurosci (2000) 5.53
Mitochondria and calcium ion transport. Biochem J (1970) 5.16
Energy-linked ion movements in mitochondrial systems. Adv Enzymol Relat Areas Mol Biol (1967) 5.10
Protonic conductance across phospholipid bilayer membranes induced by uncoupling agents for oxidative phosphorylation. Proc Natl Acad Sci U S A (1968) 4.97
Six unidentified reading frames of human mitochondrial DNA encode components of the respiratory-chain NADH dehydrogenase. Nature (1985) 4.96
Ubisemiquinone is the electron donor for superoxide formation by complex III of heart mitochondria. Arch Biochem Biophys (1985) 4.59
Production of superoxide radicals and hydrogen peroxide by NADH-ubiquinone reductase and ubiquinol-cytochrome c reductase from beef-heart mitochondria. Arch Biochem Biophys (1977) 3.40
The pathways of glutamate and glutamine oxidation by tumor cell mitochondria. Role of mitochondrial NAD(P)+-dependent malic enzyme. J Biol Chem (1984) 2.82
Sites of inhibition of mitochondrial electron transport in macrophage-injured neoplastic cells. J Cell Biol (1982) 2.54
The effect of 2,4-dinitrophenol on the electrical resistance of phospholipid bilayer membranes. Biochem Biophys Res Commun (1966) 2.53
Flavoproteins of the mitochondrial respiratory chain. Proc Natl Acad Sci U S A (1967) 2.40
Psychoeducational profile of the 22q11.2 microdeletion: A complex pattern. J Pediatr (1999) 2.38
Treatment of inborn errors of urea synthesis: activation of alternative pathways of waste nitrogen synthesis and excretion. N Engl J Med (1982) 2.34
A survey of the interaction of calcium ions with mitochondria from different tissues and species. Biochem J (1971) 2.34
Neurologic outcome in children with inborn errors of urea synthesis. Outcome of urea-cycle enzymopathies. N Engl J Med (1984) 2.25
Binary system for regulating transgene expression in mice: targeting int-2 gene expression with yeast GAL4/UAS control elements. Proc Natl Acad Sci U S A (1991) 2.16
L-lactate transport in Ehrlich ascites-tumour cells. Biochem J (1976) 1.94
Transport of ornithine and citrulline across the mitochondrial membrane. J Biol Chem (1973) 1.91
URF6, last unidentified reading frame of human mtDNA, codes for an NADH dehydrogenase subunit. Science (1986) 1.87
Stoichiometry of vectorial H+ movements coupled to electron transport and to ATP synthesis in mitochondria. Proc Natl Acad Sci U S A (1978) 1.85
The purification and properties of myo-inositol monophosphatase from bovine brain. Biochem J (1988) 1.80
Inhibition of PC12 cell redox activity is a specific, early indicator of the mechanism of beta-amyloid-mediated cell death. Proc Natl Acad Sci U S A (1994) 1.79
Creatine kinase of rat heart mitochondria. Coupling of creatine phosphorylation to electron transport. J Biol Chem (1973) 1.79
Cutaneous lymphoproliferation and lymphomas in interleukin 7 transgenic mice. J Exp Med (1993) 1.76
The cytoskeleton of digitonin-treated rat hepatocytes. Proc Natl Acad Sci U S A (1980) 1.74
Cognitive and behavior profile of preschool children with chromosome 22q11.2 deletion. Am J Med Genet (1999) 1.71
Genotype spectrum of ornithine transcarbamylase deficiency: correlation with the clinical and biochemical phenotype. Am J Med Genet (2000) 1.71
O2 solubility in aqueous media determined by a kinetic method. Anal Biochem (1985) 1.71
Risk of serious illness in heterozygotes for ornithine transcarbamylase deficiency. J Pediatr (1986) 1.70
Mitochondrial respiratory control. Evidence against the regulation of respiration by extramitochondrial phosphorylation potentials or by [ATP]/[ADP] ratios. J Biol Chem (1982) 1.69
Regulation of Ca2+ release from mitochondria by the oxidation-reduction state of pyridine nucleotides. Proc Natl Acad Sci U S A (1978) 1.67
Specific labelling of a constituent polypeptide of bovine heart mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone reductase by the inhibitor diphenyleneiodonium. Biochem J (1977) 1.66
Biochemical abnormalities and excitotoxicity in Huntington's disease brain. Ann Neurol (1999) 1.63
Long-chain acyl coenzyme A dehydrogenase deficiency: an inherited cause of nonketotic hypoglycemia. Pediatr Res (1985) 1.56
In vitro and in vivo inhibition of inositol monophosphatase by the bisphosphonate L-690,330. J Neurochem (1993) 1.56
Role of phosphate and other proton-donating anions in respiration-coupled transport of Ca2+ by mitochondria. Proc Natl Acad Sci U S A (1974) 1.51
Cerebral dysfunction in asymptomatic carriers of ornithine transcarbamylase deficiency. N Engl J Med (1980) 1.49
Stoichiometric relationship between energy-dependent proton ejection and electron transport in mitochondria. Proc Natl Acad Sci U S A (1976) 1.45
Characterization of the atractyloside-sensitive adenine nucleotide transport system in rat liver mitochondria. J Biol Chem (1968) 1.44
3-((4-(4-Chlorophenyl)piperazin-1-yl)-methyl)-1H-pyrrolo-2,3-b-pyridine: an antagonist with high affinity and selectivity for the human dopamine D4 receptor. J Med Chem (1996) 1.44
Mice expressing both B7-1 and viral glycoprotein on pancreatic beta cells along with glycoprotein-specific transgenic T cells develop diabetes due to a breakdown of T-lymphocyte unresponsiveness. Proc Natl Acad Sci U S A (1994) 1.43
Deficiency of the iron-sulfur clusters of mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase (complex I) in an infant with congenital lactic acidosis. J Clin Invest (1984) 1.42
The structure and subunit composition of the particulate NADH-ubiquinone reductase of bovine heart mitochondria. Biochem J (1976) 1.42
Prolonged metabolic correction in adult ornithine transcarbamylase-deficient mice with adenoviral vectors. J Biol Chem (1996) 1.41
Cloning and expression of bovine brain inositol monophosphatase. J Biol Chem (1990) 1.41
Regulation of free Ca2+ by liver mitochondria and endoplasmic reticulum. J Biol Chem (1980) 1.41
High affinity and low affinity binding of Ca++ by rat liver mitochondria. J Biol Chem (1969) 1.40
Stoichiometry of H+ ejection and Ca2+ uptake coupled to electron transport in rat heart mitochondria. J Biol Chem (1978) 1.39
Comparative studies on mitochondria isolated from neuron-enriched and glia-enriched fractions of rabbit and beef brain. J Cell Biol (1970) 1.35
Role of the beta subunit in determining the pharmacology of human gamma-aminobutyric acid type A receptors. Mol Pharmacol (1993) 1.34
Cognitive development in children with inborn errors of urea synthesis. Acta Paediatr Jpn (1988) 1.34
The interaction between mitochondrial NADH-ubiquinone oxidoreductase and ubiquinol-cytochrome c oxidoreductase. Evidence for stoicheiometric association. Biochem J (1978) 1.34
Amino acid acylation: a mechanism of nitrogen excretion in inborn errors of urea synthesis. Science (1980) 1.33
Cardioprotective effects of 70-kDa heat shock protein in transgenic mice. Proc Natl Acad Sci U S A (1996) 1.32
Regulated release of Ca2+ from respiring mitochondria by Ca2+/2H+ antiport. J Biol Chem (1979) 1.31
Lithium and the phosphoinositide cycle: an example of uncompetitive inhibition and its pharmacological consequences. Trends Pharmacol Sci (1991) 1.29
Behavior management of feeding disturbances in urea cycle and organic acid disorders. J Pediatr (1987) 1.29
Cytochrome bd biosynthesis in Escherichia coli: the sequences of the cydC and cydD genes suggest that they encode the components of an ABC membrane transporter. Mol Microbiol (1993) 1.29
Energy-linked synthesis and decay of membrane proteins in isolated rat liver mitochondria. Biochemistry (1966) 1.28
Ca 2+ transport activity in mitochondria from some plant tissues. Arch Biochem Biophys (1973) 1.27
The role of phospholipids in the reduction of ubiquinone analogues by the mitochondrial reduced nicotinamide-adenine dinucleotide-ubiquinone oxidoreductase complex. Biochem J (1978) 1.27
Partial resolution of the enzymes catalyzing oxidative phosphorylation. 28. The reconstitution of the first site of energy conservation. J Biol Chem (1973) 1.26
Isolation of mitochondria from ascites tumor cells permeabilized with digitonin. Anal Biochem (1984) 1.26
The affinity of mitochondrial oxidative phosphorylation mechanisms for phosphate and adenosine diphosphate. Proc Natl Acad Sci U S A (1967) 1.24
Calcium phosphate granules in the hepatopancreas of the blue crab Callinectes sapidus. J Cell Biol (1974) 1.23
cDNA cloning of human and rat brain myo-inositol monophosphatase. Expression and characterization of the human recombinant enzyme. Biochem J (1992) 1.23
The neuronal membrane. Proc Natl Acad Sci U S A (1968) 1.23
[3H]L-655,708, a novel ligand selective for the benzodiazepine site of GABAA receptors which contain the alpha 5 subunit. Neuropharmacology (1996) 1.21
Proton translocation stoichiometry of cytochrome oxidase: use of a fast-responding oxygen electrode. Proc Natl Acad Sci U S A (1982) 1.21
Interaction of Ca2+ with blowfly flight muscle mitochondria. J Biol Chem (1971) 1.20
Super-stoichiometric ratios between ion movements and electron transport in rat liver mitochondria. J Biol Chem (1967) 1.20
NADH-ubiquinone oxidoreductase. Biochim Biophys Acta (1976) 1.20
The atractyloside-sensitive nucleotide binding site in a membrane preparation from rat liver mitochondria. J Biol Chem (1968) 1.19
Atomic structure of intracellular amorphous calcium phosphate deposits. Proc Natl Acad Sci U S A (1975) 1.19
Stoichiometric relationships in mitochondrial accumulation of calcium and phosphate supported by hydrolysis of adenosine triphosphate. J Biol Chem (1966) 1.18
New gene family defined by MORC, a nuclear protein required for mouse spermatogenesis. Hum Mol Genet (1999) 1.18
Re-evaluation of the H+/site ratio of mitochondrial electron transport with the oxygen pulse technique. J Biol Chem (1976) 1.18
Mechanism of inositol monophosphatase, the putative target of lithium therapy. Proc Natl Acad Sci U S A (1994) 1.18
Aspects of energy-linked calcium accumulation by rat heart mitochondria. J Biol Chem (1975) 1.18
Stoichiometry of H+ ejection during respiration-dependent accumulation of Ca2+ by rat liver mitochondria. J Biol Chem (1976) 1.17
Cerebral palsy. Pediatr Clin North Am (1993) 1.16
Ca2+ metabolism in yeast cells and mitochondria. Biochim Biophys Acta (1970) 1.15
GABAA receptor subtypes immunopurified from rat brain with alpha subunit-specific antibodies have unique pharmacological properties. Neuron (1991) 1.13
Transport and accumulation of calcium in mitochondria. Ann N Y Acad Sci (1978) 1.13
Action of the antitumor and antispermatogenic agent lonidamine on electron transport in Ehrlich ascites tumor mitochondria. Arch Biochem Biophys (1983) 1.13
The interaction of La 3+ with mitochondria in relation to respiration-coupled Ca 2+ transport. Arch Biochem Biophys (1971) 1.12
Respiration-dependent transport of carbon dioxide into rat liver mitochondria. Biochemistry (1973) 1.11
The kinetics and inhibition of the adenosine diphosphate-adenosine triphosphate exchange catalyzed by purified mitochondrial nucleoside diphosphokinase. J Biol Chem (1967) 1.11
An analysis of the polypeptide composition of bovine heart mitochondrial NADH-ubiquinone oxidoreductase by two-dimensional polyacrylamide-gel electrophoresis. Biochem J (1979) 1.11
EPR studies of iron-sulfur clusters in isolated subunits and subfractions of NADH-ubiquinone oxidoreductase. J Biol Chem (1985) 1.10
The interaction between mitochondrial NADH-ubiquinone oxidoreductase and ubiquinol-cytochrome c oxidoreductase. Restoration of ubiquinone-pool behaviour. Biochem J (1978) 1.10
Evaluation of the H+/site ratio of mitochondrial electron transport from rate measurements. J Biol Chem (1976) 1.10
The organization of NADH dehydrogenase polypeptides in the inner mitochondrial membrane. Biochem J (1980) 1.10
The intra-mitochondrial localization of flavoproteins previously assigned to the respiratory chain. Eur J Biochem (1969) 1.09