Published in Biochem J on January 01, 1970
The regulation of triglyceride synthesis and fatty acid synthesis in rat epididymal adipose tissue. Biochem J (1970) 2.10
The regulation of glyceride synthesis in isolated white-fat cells. The effects of palmitate and lipolytic agents. Biochem J (1972) 1.52
The Effect of Exogenous Nicotinamide Adenine Dinucleotide on the Oxidation of Nicotinamide Adenine Dinucleotide-linked Substrates by Isolated Plant Mitochondria. Plant Physiol (1974) 1.50
The molecular basis for the two different clinical presentations of classical pyruvate carboxylase deficiency. Am J Hum Genet (1984) 1.12
Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia. J Clin Invest (1986) 1.07
The regulation of glyceride synthesis in isolated white-fat cells. The effects of acetate, pyruvate, lactate, palmitate, electron-acceptors, uncoupling agents and oligomycin. Biochem J (1972) 1.07
Mitochondrial respiration in subcutaneous and visceral adipose tissue from patients with morbid obesity. J Physiol (2010) 1.06
The role of the cytoplasmic redox potential in the control of fatty acid synthesis from glucose, pyruvate and lactate in white adipose tissue. Biochem J (1970) 1.05
The regulation of glutamate metabolism by tricarboxylic acid-cycle activity in rat brain mitochondria. Biochem J (1978) 0.90
Studies on the conversion of pyruvate into fatty acids in white adipose tissue. Effects of insulin, alloxan-diabetes and starvation. Biochem J (1971) 0.87
Protein measurement with the Folin phenol reagent. J Biol Chem (1951) 1743.91
METABOLISM OF ISOLATED FAT CELLS. I. EFFECTS OF HORMONES ON GLUCOSE METABOLISM AND LIPOLYSIS. J Biol Chem (1964) 21.04
The redox state of free nicotinamide-adenine dinucleotide in the cytoplasm and mitochondria of rat liver. Biochem J (1967) 12.40
An electron-transport system associated with the outer membrane of liver mitochondria. A biochemical and morphological study. J Cell Biol (1967) 12.04
Phosphorylation coupled to oxidation of dihydrodiphosphopyridine nucleotide. J Biol Chem (1951) 4.51
INFLUENCE OF THYROID HORMONES ON L-ALPHA-GLYCEROPHOSPHATE DEHYDROGENASES AND OTHER DEHYDROGENASES IN VARIOUS ORGANS OF THE RAT. J Biol Chem (1965) 2.92
STUDIES ON THE METABOLISM OF ADIPOSE TISSUE. XV. AN EVALUATION OF THE MAJOR PATHWAYS OF GLUCOSE CATABOLISM AS INFLUENCED BY INSULIN AND EPINEPHRINE. J Biol Chem (1964) 2.89
Steady state equilibria of some DPN-linked reactions and the oxidation/reduction state of the DPN/DPNH system in the cytoplasmatic compartment of liver cells in vivo. Biochem Biophys Res Commun (1961) 1.46
Studies on the metabolism of adipose tissue. XIX. An evaluation of the major pathways of glucose catabolism as influenced by acetate in the presence of insulin. J Biol Chem (1966) 1.33
KINETIC STUDIES OF GLUTAMIC OXALOACETIC TRANSAMINASE ISOZYMES. Biochemistry (1964) 1.30
The relation between intra- and extramitochondrial pyridine nucleotides. J Biol Chem (1961) 1.25
The aerobic oxidation of reduced diphosphopyridine nucleotide formed by glycolysis in Ehrlich ascites-tumour cells. Biochim Biophys Acta (1962) 0.96
Lactic acidemia and bradyarrhythmia in a child sedated with propofol. Crit Care Med (1998) 3.71
Approaches to DNA mutagenesis: an overview. Anal Biochem (1997) 3.09
Mitochondria, oxygen free radicals, disease and ageing. Trends Biochem Sci (2000) 3.04
A bovine albumin peptide as a possible trigger of insulin-dependent diabetes mellitus. N Engl J Med (1992) 2.86
The inhibition of malate, tricarboxylate and oxoglutarate entry into mitochondria by 2-n-butylmalonate. Biochem Biophys Res Commun (1967) 2.64
Heteroplasmic mtDNA mutation (T----G) at 8993 can cause Leigh disease when the percentage of abnormal mtDNA is high. Am J Hum Genet (1992) 2.41
The control of fatty acid and triglyceride synthesis in rat epididymal adipose tissue. Roles of coenzyme A derivatives, citrate and L-glycerol 3-phosphate. Biochem J (1968) 2.37
Postoperative hyponatremia despite near-isotonic saline infusion: a phenomenon of desalination. Ann Intern Med (1997) 2.33
Mitochondrial complex I deficiency leads to increased production of superoxide radicals and induction of superoxide dismutase. J Clin Invest (1996) 2.20
Encoding of anisotropic diffusion with tetrahedral gradients: a general mathematical diffusion formalism and experimental results. Magn Reson Med (1996) 2.20
Renal tubular acidosis and osteopetrosis with carbonic anhydrase II deficiency: pathogenesis of impaired acidification. Pediatr Nephrol (1997) 2.16
Quantitative analysis of amino acid oxidation and related gluconeogenesis in humans. Physiol Rev (1992) 2.12
Penetration of the mitochondrial membrane by glutamate and aspartate. Biochem Biophys Res Commun (1967) 1.99
Penetration of the mitochondrial membrane by tricarboxylic acid anions. Biochem Soc Symp (1968) 1.91
A rapid and reliable DNA preparation method for screening a large number of yeast clones by polymerase chain reaction. Nucleic Acids Res (1995) 1.84
Mutations in the X-linked pyruvate dehydrogenase (E1) alpha subunit gene (PDHA1) in patients with a pyruvate dehydrogenase complex deficiency. Hum Mutat (2000) 1.75
Hypokalaemia and paralysis. QJM (2001) 1.72
Metabolic acidosis in the alcoholic: a pathophysiologic approach. Metabolism (1983) 1.70
Hyperglycemia-induced hyponatremia: metabolic considerations in calculation of serum sodium depression. Can Med Assoc J (1975) 1.66
Importance of timing of risk factors for cerebral oedema during therapy for diabetic ketoacidosis. Arch Dis Child (2003) 1.63
The sensitivity of dicarboxylate anion exchange reactions o transport inhibitors in rat-liver mitochondria. Biochim Biophys Acta (1970) 1.62
Mitochondria, oxygen free radicals, and apoptosis. Am J Med Genet (2001) 1.61
Reverse micelles as hosts for proteins and small molecules. Biochim Biophys Acta (1988) 1.58
A biochemically distinct form of cytochrome oxidase (COX) deficiency in the Saguenay-Lac-Saint-Jean region of Quebec. Am J Hum Genet (1993) 1.57
Cancer in patients receiving dialysis. Br Med J (1980) 1.52
How to select optimal maintenance intravenous fluid therapy. QJM (2003) 1.51
Glue-sniffing and distal renal tubular acidosis: sticking to the facts. J Am Soc Nephrol (1991) 1.50
Acidosis in a patient with cholera: a need to redefine concepts. QJM (2004) 1.47
An additional role for insulin in the control of fatty acid synthesis independent of glucose transport. Can J Biochem (1970) 1.47
A modified classification of metabolic acidosis: a pathophysiologic approach. Nephron (1992) 1.45
Sites of ammonia addition to tubular fluid in rats with chronic metabolic acidosis. Kidney Int (1981) 1.45
Treatment of acute hyponatremia: ensuring the excretion of a predictable amount of electrolyte-free water. Crit Care Med (2000) 1.44
Severe hypokalaemia in a Chinese male. QJM (2002) 1.42
The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis. Pediatr Res (1980) 1.41
Clinical, metabolic, and genetic aspects of cytochrome C oxidase deficiency in Saguenay-Lac-Saint-Jean. Am J Hum Genet (1993) 1.40
Isolation and sequence determination of cDNA clones for porcine and human lipoamide dehydrogenase. Homology to other disulfide oxidoreductases. J Biol Chem (1987) 1.40
The patient with a severe degree of metabolic acidosis: a deductive analysis. QJM (2006) 1.39
Superoxides from mitochondrial complex III: the role of manganese superoxide dismutase. Free Radic Biol Med (2000) 1.37
Isolation of a full-length complementary DNA coding for human E1 alpha subunit of the pyruvate dehydrogenase complex. J Biol Chem (1988) 1.33
Barth syndrome: clinical observations and genetic linkage studies. Am J Med Genet (1994) 1.31
Studies on the pathogenesis of type I (distal) renal tubular acidosis as revealed by the urinary PCO2 tensions. J Clin Invest (1974) 1.30
An analysis of the reflex systemic vasodilator response elicited by lung inflation in the dog. J Physiol (1968) 1.29
Expression of mtDNA and nDNA encoded respiratory chain proteins in chemically and genetically-derived Rho0 human fibroblasts: a comparison of subunit proteins in normal fibroblasts treated with ethidium bromide and fibroblasts from a patient with mtDNA depletion syndrome. Biochim Biophys Acta (1997) 1.29
Regulation of glycolysis and L-glycerol 3-phosphate concentration in rat epididymal adipose tissue in vitro. Role of phosphofructokinase. Biochem J (1969) 1.27
[3H]biotin-labeled proteins in cultured human skin fibroblasts from patients with pyruvate carboxylase deficiency. J Biol Chem (1983) 1.27
Familial Hibernian fever. Q J Med (1982) 1.26
Iron metabolism in the anaemia of chronic renal failure. Effects of dialysis and of parenteral iron. Br Med J (1969) 1.26
Site-specific incorporation of nitroxide spin-labels into internal sites of the TAR RNA; structure-dependent dynamics of RNA by EPR spectroscopy. J Am Chem Soc (2001) 1.23
Agonal phase, ischaemic times, and renal vascular abnormalities and outcome of cadaver kidney transplants. Br Med J (1975) 1.22
A novel syndrome affecting multiple mitochondrial functions, located by microcell-mediated transfer to chromosome 2p14-2p13. Am J Hum Genet (2001) 1.22
Factors that control the effect of pH on glycolysis in leukocytes. J Biol Chem (1969) 1.20
Motions of short DNA duplexes: an analysis of DNA dynamics using an EPR-active probe. Biochemistry (1993) 1.20
Interfacial membrane docking of cytosolic phospholipase A2 C2 domain using electrostatic potential-modulated spin relaxation magnetic resonance. Proc Natl Acad Sci U S A (1999) 1.18
Sequence-dependent dynamics of duplex DNA: the applicability of a dinucleotide model. Biophys J (2002) 1.18
The transtubular potassium concentration in patients with hypokalemia and hyperkalemia. Am J Kidney Dis (1990) 1.17
Analysis of double-helix motions with spin-labeled probes: binding geometry and the limit of torsional elasticity. J Mol Biol (1980) 1.16
The mitochondrial DNA mutation at 8993 associated with NARP slows the rate of ATP synthesis in isolated lymphoblast mitochondria. Biochem Biophys Res Commun (1993) 1.16
Sequence- and structure-dependent DNA base dynamics: synthesis, structure, and dynamics of site and sequence specifically spin-labeled DNA. Biochemistry (1989) 1.16
Simultaneous detection of mitochondrial respiratory chain activity and reactive oxygen in digitonin-permeabilized cells using flow cytometry. Cytometry (2000) 1.14
The effects of graded doses of phenoxybenzamine on the vascular and capsular responses of the isolated, blood-perfused dogs spleen to sympathetic nerve stimulation and catecholamines. Arch Int Pharmacodyn Ther (1969) 1.12
A genomewide linkage-disequilibrium scan localizes the Saguenay-Lac-Saint-Jean cytochrome oxidase deficiency to 2p16. Am J Hum Genet (2001) 1.12
The molecular basis for the two different clinical presentations of classical pyruvate carboxylase deficiency. Am J Hum Genet (1984) 1.12
Defective intramitochondrial NADH oxidation in skin fibroblasts from an infant with fatal neonatal lacticacidemia. Am J Hum Genet (1985) 1.11
3-Mercaptopicolinic acid, a preferential inhibitor of the cytosolic phosphoenolpyruvate carboxykinase. FEBS Lett (1975) 1.10
Development of a test to evaluate the transtubular potassium concentration gradient in the cortical collecting duct in vivo. Miner Electrolyte Metab (1986) 1.10
Using nitroxide spin labels. How to obtain T1e from continuous wave electron paramagnetic resonance spectra at all rotational rates. Biophys J (1993) 1.09
Structure, functioning, and assembly of the ATP synthase in cells from patients with the T8993G mitochondrial DNA mutation. Comparison with the enzyme in Rho(0) cells completely lacking mtdna. J Biol Chem (2000) 1.09
Anorexia nervosa and chronic renal insufficiency: a prescription for disaster. QJM (2004) 1.09
3-Oxothiolase activities and [14C]-2-methylbutanoic acid incorporation in cultured fibroblasts from 13 cases of suspected 3-oxothiolase deficiency. Pediatr Res (1990) 1.09
Factors affecting the kinetics and equilibrium of exchange reactions of the citrate-transporting system of rat liver mitochondria. J Biol Chem (1971) 1.08
Sequence-dependent dynamics in duplex DNA. Biophys J (2000) 1.07
DNA packing in single crystals inferred from freeze-fracture-etch replicas. J Mol Biol (1976) 1.07
Respiratory chain defects in the mitochondria of cultured skin fibroblasts from three patients with lacticacidemia. J Clin Invest (1986) 1.07
Effect of 2-methylcitrate on citrate metabolism: implications for the management of patients with propionic acidemia and methylmalonic aciduria. Pediatr Res (1975) 1.07
Interpretation of the serum potassium concentration in metabolic acidosis. Clin Invest Med (1979) 1.06
Monitoring DNA dynamics using spin-labels with different independent mobilities. Biochemistry (1995) 1.05
Flexibility of duplex DNA on the submicrosecond timescale. Biophys J (1999) 1.05
The role of the cytoplasmic redox potential in the control of fatty acid synthesis from glucose, pyruvate and lactate in white adipose tissue. Biochem J (1970) 1.05
An additional mitochondrial tRNA(Ile) point mutation (A-to-G at nucleotide 4295) causing hypertrophic cardiomyopathy. Hum Mutat (1996) 1.05
Characterization of cytochrome-c oxidase mutants in human fibroblasts. FEBS Lett (1988) 1.04
Brain swelling after dialysis: old urea or new osmoles? Am J Kidney Dis (1996) 1.04
Maternally inherited hypertrophic cardiomyopathy due to a novel T-to-C transition at nucleotide 9997 in the mitochondrial tRNA(glycine) gene. Am J Hum Genet (1994) 1.03
Reduction in adipocyte ATP by lipolytic agents: relation to intracellular free fatty acid accumulation. J Lipid Res (1971) 1.02
New clinical approach to evaluate disorders of potassium excretion. Miner Electrolyte Metab (1986) 1.02
Hypokalaemia and paralysis. QJM (2003) 1.01
Clinical presentation of mitochondrial respiratory chain defects in NADH-coenzyme Q reductase and cytochrome oxidase: clues to pathogenesis of Leigh disease. J Pediatr (1987) 1.01
Docking phospholipase A2 on membranes using electrostatic potential-modulated spin relaxation magnetic resonance. Science (1998) 1.01
3-Hydroxy-3-methylglutaric aciduria. J Neurogenet (1984) 1.00
The effects of 2-ethylcitrate and tricarballylate on citrate transport in rat liver mitochondria and fatty acid synthesis in rat white adipose tissue. Eur J Biochem (1970) 1.00
Familial cardiomyopathy with cataracts and lactic acidosis: a defect in complex I (NADH-dehydrogenase) of the mitochondria respiratory chain. Pediatr Res (1996) 0.99
Deficiency of dihydrolipoyl dehydrogenase (a component of the pyruvate and alpha-ketoglutarate dehydrogenase complexes): a cause of congenital chronic lactic acidosis in infancy. Pediatr Res (1977) 0.99
NADH-coenzyme Q reductase (complex I) deficiency: heterogeneity in phenotype and biochemical findings. J Inherit Metab Dis (1996) 0.98
Lactic acidosis due to pyruvate carboxylase deficiency. J Inherit Metab Dis (1981) 0.98
Lactic acidaemia. J Inherit Metab Dis (1984) 0.98
The 8993 mtDNA mutation: heteroplasmy and clinical presentation in three families. Eur J Hum Genet (1994) 0.97
Urine electrolytes in the assessment of extracellular fluid volume contraction. Am J Nephrol (1989) 0.97