(Q72929225)

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

30 October 2019

title

Problems in the congenital lactic acidoses (English)

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

30 October 2019

Problems in the Congenital Lactic Acidoses (English)

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

15 September 2023

author name string

J. V. Leonard

1

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?resulttype=core&format=json&query=SRC%3AMED%20AND%20EXT_ID%3A6280937

15 September 2023

language of work or name

English

1 reference

National Center for Biotechnology Information

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/esummary.fcgi?db=pubmed&retmode=json&id=6280937

15 September 2023

publication date

1 January 1982

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

full work available at URL

30 October 2019

https://api.wiley.com/onlinelibrary/tdm/v1/articles/10.1002%2F9780470720691.ch19

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

https://onlinelibrary.wiley.com/doi/pdf/10.1002/9780470720691.ch19

15 September 2023

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Ciba Foundation Symposium

15 September 2023

published in

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

30 October 2019

volume

87

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

30 October 2019

page(s)

340-356

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

Pyruvate Carboxylase Deficiency and Lactic Acidosis in a Retarded Child without Leigh's Disease

30 October 2019

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Pyruvate metabolism in Friedreich's ataxia

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Studies on cultured fibroblasts from patients with defects of biotin‐dependent carboxylation

15 September 2023

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A defect in pyruvate decarboxylase in a child with an intermittent movement disorder

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Clinical Studies of a Patient With Pyruvate Decarboxylase Deficiency

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An inherited defect affecting the tricarboxylic acid cycle in a patient with congenital lactic acidosis

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Low Activities of the Pyruvate and Oxoglutarate Dehydrogenase Complexes in Five Patients with Friedreich's Ataxia

15 September 2023

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Rapid diagnosis of pyruvate and ketoglutarate dehydrogenase deficiencies in platelet-enriched preparations from blood

15 September 2023

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Sensitivity to carbohydrate in a patient with familial intermittent lactic acidosis and pyruvate dehydrogenase deficiency

15 September 2023

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Fatal infhntile mitochondrial myopathy and renal dysfunction due to cytochrome-c-oxidase deficiency

15 September 2023

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The Clinical and Biochemical Implications of Pyruvate Carboxylase Deficiency

15 September 2023

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Absence of pyruvate decarboxylase activity in man: a cause of congenital lactic acidosis

15 September 2023

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The intravenous L-alanine tolerance test as a means for investigating gluconeogenesis.

15 September 2023

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Urinary lactate excretion in normal children and in children with enzyme defects of carbohydrate metabolism

15 September 2023

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Effect of oral alanine on blood beta-hydroxybutyrate and plasma glucose, insulin, free fatty acids, and growth hormone in normal and diabetic subjects.

15 September 2023

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Biochemical studies and therapy in subacute necrotizing encephalomyelopathy (Leigh's syndrome)

15 September 2023

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Studies on pyruvate carboxylase from cultured human fibroblasts and amniotic fluid cells

15 September 2023

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Lactate metabolism. Studies of a child with a serious congenital deviation

15 September 2023

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Leigh's encephalomyelopathy: an inborn error of gluconeogenesis

15 September 2023

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Two cases of phosphoenolpyruvate carboxykinase deficiency

15 September 2023

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Pyruvate dehydrogenase deficiency in spinocerebellar degenerations

15 September 2023

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Pyruvate oxidation in neuromuscular diseases. Evidence of a genetic defect in two families with the clinical syndrome of Friedreich's ataxia.

15 September 2023

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Blood sampling technique for lactate and pyruvate estimation in children

15 September 2023

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Abnormal Pyruvate and α-Ketoglutarate Dehydrogenase Complexes in a Patient with Lactic Acidemia

15 September 2023

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Inherited disorders of 3-methylcrotonyl CoA carboxylation

15 September 2023

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Pyruvate Dehydrogenase, Lipoamide Dehydrogenase and Citrate Synthase Activity in Fibroblasts from Patients with Friedreich's and Charlevoix-Saguenay Ataxia

15 September 2023

43

1 reference

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Proceedings: L-glutamine therapy in Leigh's encephalomyelopathy

15 September 2023

44

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A mitochondrial myopathy characterized by a deficiency in reducible cytochrome b.

15 September 2023

45

1 reference

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A mitochondrial myopathy with a deficiency of respiratory chain NADH-CoQ reductase activity

15 September 2023

46

1 reference

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The effect of alanine on glucagon secretion

15 September 2023

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Citrate treatment in a patient with pyruvate decarboxylase deficiency

15 September 2023

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Hormonal Control of Hepatic Gluconeogenesis

15 September 2023

49

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Pyruvate carboxylase and phosphoenopyruvate carboxykinase in cultured human fibroblasts

15 September 2023

50

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Deficiency of Dihydrolipoyl Dehydrogenase (a Component of the Pyruvate and α-Ketoglutarate Dehydrogenase Complexes): A Cause of Congenital Chronic Lactic Acidosis in Infancy

15 September 2023

52

1 reference

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The genetic heterogeneity of lactic acidosis: occurrence of recognizable inborn errors of metabolism in pediatric population with lactic acidosis

15 September 2023

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1 reference

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Lipoamide dehydrogenase deficiency.

15 September 2023

55

1 reference

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Studies in type I glycogenosis of the liver. The genesis and disposition of lactate

15 September 2023

56

1 reference

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Neonatal congenital lactic acidosis with pyruvate carboxylase deficiency in two siblings

15 September 2023

57

1 reference

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Purification and properties of human liver pyruvate carboxylase

15 September 2023

58

1 reference

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INSTABILITY OF PYRUVATE-C-14 IN AQUEOUS SOLUTION AS DETECTED BY ENZYMIC ASSAY

15 September 2023

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Fructose load test—an in vivo screening test designed to assess pyruvate dehydrogenase activity and interconversion

15 September 2023

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Fatal lactic acidosis in a newborn attributable to a congenital defect of pyruvate dehydrogenase

15 September 2023

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Friedreich's ataxia: I. Normal pyruvate dehydrogenase complex activity in platelets

15 September 2023

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Hyperalaninemia with pyruvicemia in a patient suggestive of Leigh's encephalomyelopathy.

15 September 2023

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Congenital lactic acidosis due to pyruvate carboxylase deficiency: Absence of an inhibitor of TPP-ATP phosphoryl transferase

15 September 2023

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Pathogenesis of Leigh's encephalomyelopathy.

15 September 2023

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A Defect in Branched-Chain Amino Acid Metabolism in a Patient with Congenital Lactic Acidosis due to Dihydrolipoyl Dehydrogenase Deficiency

15 September 2023

66

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Hereditary mitochondrial myopathy with lactic acidemia, a De Toni-Fanconi-Debré syndrome, and a defective respiratory chain in voluntary striated muscles

15 September 2023

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Report of a Patient with Severe, Chronic Lactic Acidaemia and Pyruvate Carboxylase Deficiency

15 September 2023

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GLUCONEOGENESIS IN INFANCY AND CHILDHOOD III. Deficiency of the Extramitochondrial Form of Hepatic Phosphoenolpyruvate Carboxykinase in a Case of Persistent Neonatal Hypoglycaemia

15 September 2023

71

1 reference

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Systemic carnitine deficiency. Report of a fatal case with multisystemic manifestations

15 September 2023

72

1 reference

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Thiamine dependency in a patient with congenital lacticacidaemia due to pyruvate dehydrogenase deficiency

15 September 2023

73

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Variability of acid hydrolase activities in cultured skin fibroblasts and amniotic fluid cells

15 September 2023

75

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Preclinical diagnosis and carrier detection in ataxia associated with abnormalities of lipoamide dehydrogenase

15 September 2023

77

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Metabolic and biochemical studies in fructose 1,6-diphosphatase deficiency.

15 September 2023

79

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Chronic lactic acidosis in an adult. A new syndrome associated with an altered redox state of certain NAD-NADH coupled reactions

15 September 2023

80

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Hepatic Autophagy in Uncontrolled Experimental Diabetes and Its Relationships to Insulin and Glucagon

15 September 2023

82

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Lactic acidosis: An experimental model

15 September 2023

83

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Insulin stimulates sugar transport in giant muscle fibres of the barnacle

15 September 2023

84

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Ketoacidosis in Pancreatectomized Man

15 September 2023

85

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Importance of pituitary hormones in aetiology of diabetic ketoacidosis

15 September 2023

86

1 reference

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Regulatory mechanisms of hemoglobin oxygen affinity in acidosis and alkalosis

15 September 2023

87

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

The metabolic effects of sodium dichloroacetate in the starved rat.

15 September 2023

89

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Hemodynamic mechanisms in the development of pulmonary venous admixture (shunting)

15 September 2023

91

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Calcium ions and the regulation of NAD+-linked isocitrate dehydrogenase from the mitochondria of rat heart and other tissues

15 September 2023

95

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Acute symptomatic ketoacidosis following growth hormone administration in prolonged fasting

15 September 2023

96

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Blood lactate in the development of anxiety symptoms. A critical examination of Pitts and McClure's hypothesis and experimental study

15 September 2023

98

1 reference

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Hemodynamic effects of dehydration and metabolic acidosis in Asiatic cholera

15 September 2023

99

1 reference

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Phospholipid Methylation and Biological Signal Transmission

15 September 2023

101

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Anxiety and the Effects of Sodium Lactate Assessed Clinically and Physiologically

15 September 2023

102

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

The Effect of Simulated Metabolic Acidosis on Intracellular pH and Lactate Metabolism in the Isolated Perfused Rat Liver

15 September 2023

104

1 reference

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The Role of Metabolic Acidosis in the Pulmonary Vascular Response to Hemorrhage and Shock

15 September 2023

105

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Hormonal control of ketogenesis. Rapid activation of hepatic ketogenic capacity in fed rats by anti-insulin serum and glucagon

15 September 2023

107

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

A possible role for malonyl-CoA in the regulation of hepatic fatty acid oxidation and ketogenesis

15 September 2023

108

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

The Regulation of Contractile Activity in Muscle

15 September 2023

110

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Lactate Metabolism in Anxiety Neurosis

15 September 2023

113

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Measurement of myocardial intracellular pH in pathological states

15 September 2023

114

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Regulation of pyruvate oxidation and the conservation of glucose

15 September 2023

115

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Changes in the concentration of activation factor for phosphofructokinase in hepatocytes in response to glucose and glucagon

15 September 2023

117

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Role of glucagon, catecholamines, and growth hormone in human glucose counterregulation. Effects of somatostatin and combined alpha- and beta-adrenergic blockade on plasma glucose recovery and glucose flux rates after insulin-induced hypoglycemia

15 September 2023

118

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Pathophysiology of metabolic acidosis: effect of low pH on the hepatic uptake of lactate, pyruvate and alanine

15 September 2023

120

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Acute pulmonary hypertension in idiopathic lactic acidosis

15 September 2023

121

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Comparison of the metabolic responses to portal and peripheral infusions of insulin in diabetic dogs

15 September 2023

123

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

Mechanism of activation of pyruvate dehydrogenase by dichloroacetate and other halogenated carboxylic acids

15 September 2023

126

1 reference

https://api.crossref.org/works/10.1002/9780470720691.CH19

15 September 2023

Identifiers

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:6280937%20AND%20SRC:MED&resulttype=core&format=json

30 October 2019

1 reference