Published in J Neurochem on January 01, 1977
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Plasma acetone metabolism in the fasting human. J Clin Invest (1979) 1.21
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Outcome of pyruvate dehydrogenase deficiency treated with ketogenic diets. Studies in patients with identical mutations. Neurology (1997) 1.21
Cloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexes. Proc Natl Acad Sci U S A (1988) 1.20
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The golem of osteoporosis genetics. J Rheumatol (1998) 1.06
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Inhibition by the branched-chain 2-oxo acids of the 2-oxoglutarate dehydrogenase complex in developing rat and human brain. Biochem J (1974) 1.03
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Ethanol causes acute inhibition of carbohydrate, fat, and protein oxidation and insulin resistance. J Clin Invest (1988) 1.00
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Identification of two mutations in a compound heterozygous child with dihydrolipoamide dehydrogenase deficiency. Hum Mol Genet (1996) 0.95
Monitoring metabolic control in diabetic outpatients with glycosylated hemoglobin. Ann Intern Med (1980) 0.95
Treatment of inoperable glucagonoma with the long-acting somatostatin analogue SMS 201-995. N Engl J Med (1986) 0.95
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Nature and quantity of fuels consumed in patients with alcoholic cirrhosis. J Clin Invest (1983) 0.94
Lipogenesis in developing guinea pig liver. Mech Ageing Dev (1974) 0.94
Systemic deficiency of the first component of the pyruvate dehydrogenase complex. Pediatr Res (1987) 0.94
The newborn of diabetic rat. I. Hormonal and metabolic changes in the postnatal period. Pediatr Res (1982) 0.94
Metabolic effects of exogenous glucocorticoids in fasted man. J Clin Invest (1973) 0.94
Nucleotide sequence of a cDNA for the dihydrolipoamide acetyltransferase component of human pyruvate dehydrogenase complex. FEBS Lett (1988) 0.94
The effect of phenylpyruvate on pyruvate metabolism in rat brain. Biochem J (1972) 0.93
Development of mitochondrial pyruvate metabolism in rat brain. J Neurochem (1974) 0.93
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Long-term effects of dietary fiber on glucose tolerance and gastric emptying in noninsulin-dependent diabetic patients. Am J Clin Nutr (1983) 0.91
A deficiency of both subunits of pyruvate dehydrogenase which is not expressed in fibroblasts. Pediatr Res (1988) 0.91
Substrate, hormone, and temperature responses in males and females to a common breakfast. Metabolism (1980) 0.90
Characterization of cDNAs encoding human pyruvate dehydrogenase alpha subunit. Proc Natl Acad Sci U S A (1989) 0.90
Lipogenesis from ketone bodies in rat brain. Evidence for conversion of acetoacetate into acetyl-coenzyme A in the cytosol. Biochem J (1976) 0.90
Regulation of human growth hormone receptor gene expression by human growth hormone in a human hepatoma cell line. Mol Cell Endocrinol (1991) 0.90
Fatty acid synthesis by human adipose tissue. Metabolism (1975) 0.90
Identification of two missense mutations in a dihydrolipoamide dehydrogenase-deficient patient. Proc Natl Acad Sci U S A (1993) 0.89
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Assessment of norepinephrine secretion and production. J Lab Clin Med (1983) 0.89
Characterization of two site-specifically mutated human dihydrolipoamide dehydrogenases (His-452----Gln and Glu-457----Gln). J Biol Chem (1992) 0.89
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A dietary intervention (high carbohydrate) during the neonatal period causes islet dysfunction in rats. Am J Physiol (1999) 0.88
Deficiency of dihydrolipoamide dehydrogenase due to two mutant alleles (E340K and G101del). Analysis of a family and prenatal testing. Biochim Biophys Acta (1997) 0.88
Genetic defects in human pyruvate dehydrogenase. Ann N Y Acad Sci (1989) 0.88