(Q39504293)

12 September 2017

1 reference

title

Disorders of the pyruvate dehydrogenase complex (English)

1 reference

12 September 2017

D Stansbie

1

1 reference

PubMed ID

3018357

12 September 2017

S J Wallace

series ordinal

2

1 reference

PubMed ID

3018357

12 September 2017

C Marsac

series ordinal

3

1 reference

12 September 2017

1 January 1986

1 reference

Journal of Inherited Metabolic Disease

12 September 2017

1 reference

12 September 2017

9

1 reference

12 September 2017

2

1 reference

12 September 2017

105-119

1 reference

https://scigraph.springernature.com/pub.10.1007/bf01799447

12 September 2017

0 references

cites work

Regulation of adipose tissue pyruvate dehydrogenase by insulin and other hormones

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Pyruvate decarboxylase deficiency in subacute necrotizing encephalomyelopathy

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Subdural empyema in infants, children and adults

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Pyruvate dehydrogenase deficiency in spinocerebellar degenerations

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Errors arising from the use of [1-14C]pyruvate to measure flux through the liver pyruvate dehydrogenase complex

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Deficient Activity of Hepatic Pyruvate Dehydrogenase and Pyruvate Carboxylase in Reye's Syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Abnormal activation of pyruvate dehydrogenase in Leigh disease fibroblasts.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Enzymologic studies and therapy of Leigh's disease associated with pyruvate decarboxylase deficiency.

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

The elementary reactions of the pig heart pyruvate dehydrogenase complex. A study of the inhibition by phosphorylation

21 January 2018

1 reference

https://api.crossref.org/works/10.1007%2FBF01799447

Pyruvate dehydrogenase deficiency restricted to brain.

21 January 2018

1 reference

12 December 2020

Reduced enzyme activities in inherited ataxia

1 reference

12 December 2020

Pyruvate dehydrogenase subcomplex with lipoamide dehydrogenase deficiency in a patient with lactic acidosis and branched chain ketoaciduria

1 reference

12 December 2020

Hyper-endorphin syndrome in a child with necrotizing encephalomyelopathy

1 reference

12 December 2020

Fatal case of pyruvate dehydrogenase deficiency

1 reference

12 December 2020

Spectrophotometric measurement of pyruvate dehydrogenase complex activity in cultured human fibroblasts

1 reference

12 December 2020

The mammalian pyruvate dehydrogenase complex: structure and regulation

1 reference

12 December 2020

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

1 reference

12 December 2020

Pyruvate dehydrogenase activity is not deficient in the brain of three autopsied cases with Leigh disease (subacute necrotizing encephalomyelopathy, SNE)

1 reference

12 December 2020

Regulation of the Human Pyruvate Dehydrogenase Complex

1 reference

12 December 2020

Human muscle pyruvate dehydrogenase activity

1 reference

12 December 2020

Pyruvate Dehydrogenase Phosphatase Deficiency: A Cause of Congenital Chronic Lactic Acidosis in Infancy

1 reference

12 December 2020

Studies on pyruvate carboxylase, pyruvate decarboxylase and lipoamide dehydrogenase in subacute necrotizing encephalomyelopathy.

1 reference

12 December 2020

Clinical Studies of a Patient With Pyruvate Decarboxylase Deficiency

1 reference

12 December 2020

An improved method for the assay of platelet pyruvate dehydrogenase

1 reference

12 December 2020

An NADH-linked spectrophotometric assay for pyruvate dehydrogenase complex in crude tissue homogenates

1 reference

12 December 2020

Letter: Pyruvate decarboxylase deficiency in liver

1 reference

12 December 2020

Lactic acidosis in three sibs due to defects in both pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase complexes

1 reference

12 December 2020

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

1 reference

12 December 2020

Low immunogenicity of the common lipoamide dehydrogenase subunit (E3) of mammalian pyruvate dehydrogenase and 2-oxoglutarate dehydrogenase multienzyme complexes

1 reference

12 December 2020

Intermittent ataxia with pyruvate-decarboxylase deficiency.

1 reference

12 December 2020

Determination of pyruvate dehydrogenase in cultured human fibroblasts and amniotic fluid cells

1 reference

12 December 2020

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

1 reference

12 December 2020

Leigh's encephalomyelopathy in a patient with cytochrome c oxidase deficiency in muscle tissue

1 reference

12 December 2020

Leigh's encephalomyelopathy: an inborn error of gluconeogenesis

1 reference

12 December 2020

Central hypoventilation syndrome in pyruvate dehydrogenase complex deficiency.

1 reference

12 December 2020

Pyruvate dehydrogenase complex activity in normal and deficient fibroblasts

1 reference

12 December 2020

An improved procedure for the assay of pyruvate dehydrogenase

1 reference

12 December 2020

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

1 reference

12 December 2020

Ketonic diet in the management of pyruvate dehydrogenase deficiency

1 reference

12 December 2020

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

1 reference

12 December 2020

The effect of mycoplasma contamination on the in vitro assay of pyruvate dehydrogenase activity in cultured fibroblasts

1 reference

12 December 2020

Lactic acidemia, neurologic deterioration and carbohydrate dependence in a girl with dihydrolipoyl dehydrogenase deficiency

1 reference

12 December 2020

Methodology in evaluating pyruvate oxidation

1 reference

12 December 2020

Lipoamide dehydrogenase deficiency with primary lactic acidosis: favorable response to treatment with oral lipoic acid

1 reference

12 December 2020

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

1 reference

12 December 2020

Decreased pyruvate dehydrogenase complex activity in Huntington and Alzheimer brain

1 reference

12 December 2020

Revised assays for the investigation of congenital lactic acidosis using 14C keto acids, eliminating problems associated with spontaneous decarboxylation

1 reference

12 December 2020

Effect of mycoplasma infection on pyruvate dehydrogenase complex activity of normal and pyruvate dehydrogenase complex-deficient fibroblasts

1 reference

12 December 2020

The enzymatic acetylation of amines.

1 reference

12 December 2020

Inborn errors of pyruvate metabolism

1 reference

12 December 2020

1 reference

12 December 2020

Defective activation of the pyruvate dehydrogenase complex in subacute necrotizing encephalomyelopathy (leigh disease)

1 reference

12 December 2020

Pyruvate decarboxylating activity in extracts from pigeon liver acetone powder

1 reference

12 December 2020

A coupled fluorometric rate assay for pyruvate dehydrogenase in cultured human fibroblasts

1 reference

12 December 2020

Normal activities of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Leigh's syndrome.

1 reference

12 December 2020

Distribution of phosphate-activated glutaminase, succinic dehydrogenase, pyruvate dehydrogenase and gamma-glutamyl transpeptidase in post-mortem brain from Huntington's disease and agonal cases

1 reference

12 December 2020

Pyruvate dehydrogenase phosphate (PDHb) phosphatase activity in fibroblasts from Leigh's disease

1 reference

12 December 2020

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

1 reference

12 December 2020

Subacute necrotizing encephalomyelopathy in an infant

1 reference

12 December 2020

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

1 reference

12 December 2020

Letter: Biochemical abnormalities in Leigh's disease

1 reference

12 December 2020

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

1 reference

12 December 2020

Pyruvic and alpha-ketoglutaric oxidase of animal tissue

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF01799447

1 reference

12 September 2017

1 reference

PubMed ID

3018357