(Q36796211)

16 August 2017

1 reference

title

Biochemical assays for mitochondrial activity: assays of TCA cycle enzymes and PDHc (English)

1 reference

16 August 2017

Ann Saada Reisch

1

1 reference

PubMed ID

17445696

16 August 2017

Orly Elpeleg

series ordinal

2

1 reference

16 August 2017

1 January 2007

1 reference

16 August 2017

Methods in Cell Biology

1 reference

16 August 2017

80

1 reference

16 August 2017

199-222

1 reference

Activities of citrate synthase and NAD+-linked and NADP+-linked isocitrate dehydrogenase in muscle from vertebrates and invertebrates

16 August 2017

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Liver Disease in the Ashkenazi-Jewish Lipoamide Dehydrogenase Deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mutations in PDX1, the human lipoyl-containing component X of the pyruvate dehydrogenase-complex gene on chromosome 11p1, in congenital lactic acidosis

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Lipoamide dehydrogenase activity in lymphocytes

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Amino acid sequence homology among the 2-hydroxy acid dehydrogenases: mitochondrial and cytoplasmic malate dehydrogenases form a homologous system with lactate dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Protein components of mitochondrial DNA nucleoids in higher eukaryotes

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mutation of the fumarase gene in two siblings with progressive encephalopathy and fumarase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Succinate dehydrogenase deficiency in human

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Aconitase couples metabolic regulation to mitochondrial DNA maintenance

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Increased synthesis and decreased stability of mitochondrial translation products in yeast as a result of loss of mitochondrial (NAD(+))-dependent isocitrate dehydrogenase.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Structural and genetic relationships between cytosolic and mitochondrial isoenzymes

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Deficiency of the ADP-forming succinyl-CoA synthase activity is associated with encephalomyopathy and mitochondrial DNA depletion

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Variability of clinical presentation in fumarate hydratase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Lipoamide dehydrogenase deficiency: a new cause for recurrent myoglobinuria

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Metabolic disruption in Drosophila bang-sensitive seizure mutants

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

A mitochondrial encephalomyopathy: the first case with an established defect at the level of coenzyme Q.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Interaction between succinyl CoA synthetase and the heme-biosynthetic enzyme ALAS-E is disrupted in sideroblastic anemia

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Branched-chain 2-oxo acid dehydrogenase interferes with the measurement of the activity and activity state of hepatic pyruvate dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Reciprocal control of RNA-binding and aconitase activity in the regulation of the iron-responsive element binding protein: role of the iron-sulfur cluster

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mitochondrial myopathy with succinate dehydrogenase and aconitase deficiency. Abnormalities of several iron-sulfur proteins

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

A simple spectrophotometric assay for fumarate hydratase in crude tissue extracts

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Clinical and genetic spectrum of pyruvate dehydrogenase deficiency: dihydrolipoamide acetyltransferase (E2) deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Regulation of pyruvate dehydrogenase complex activity by reversible phosphorylation

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

HIF overexpression correlates with biallelic loss of fumarate hydratase in renal cancer: novel role of fumarate in regulation of HIF stability

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Control of mitochondrial redox balance and cellular defense against oxidative damage by mitochondrial NADP+-dependent isocitrate dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Purification and properties of tuna supernatant and mitochondrial malate dehydrogenases

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Localization and characterization of the mitochondrial isoform of the nucleoside diphosphate kinase in the pancreatic beta cell: evidence for its complexation with mitochondrial succinyl-CoA synthetase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Pyruvate dehydrogenase phosphatase deficiency: identification of the first mutation in two brothers and restoration of activity by protein complementation

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Multiple cellular consequences of isocitrate dehydrogenase isozyme dysfunction

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Global transcription analysis of Krebs tricarboxylic acid cycle mutants reveals an alternating pattern of gene expression and effects on hypoxic and oxidative genes

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Molecular cloning and deduced amino acid sequences of the alpha- and beta- subunits of mammalian NAD(+)-isocitrate dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

A novel mutation in the dihydrolipoamide dehydrogenase E3 subunit gene (DLD) resulting in an atypical form of alpha-ketoglutarate dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Friedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Disorders of pyruvate metabolism and the tricarboxylic acid cycle.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Fumarate hydratase enzyme activity in lymphoblastoid cells and fibroblasts of individuals in families with hereditary leiomyomatosis and renal cell cancer.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Accumulation of Krebs cycle intermediates and over-expression of HIF1alpha in tumours which result from germline FH and SDH mutations

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Yeast aconitase in two locations and two metabolic pathways: seeing small amounts is believing

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Defects in the E2 lipoyl transacetylase and the X-lipoyl containing component of the pyruvate dehydrogenase complex in patients with lactic acidemia

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Inborn errors of complex II--unusual human mitochondrial diseases

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Biochemical and molecular investigations in respiratory chain deficiencies

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Inborn errors of the Krebs cycle: a group of unusual mitochondrial diseases in human

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Deoxyribonucleotides and disorders of mitochondrial DNA integrity.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

ATP synthesis in lipoamide dehydrogenase deficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mutant mitochondrial thymidine kinase in mitochondrial DNA depletion myopathy

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Folding of fumarase during mitochondrial import determines its dual targeting in yeast

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Enzyme levels of the NADH shuttle systems: measurements in isolated muscle fibres from humans of differing physical activity

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Subcellular distribution of isocitrate dehydrogenase in early and term human placenta

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Optimized spectrophotometric assay for the completely activated pyruvate dehydrogenase complex in fibroblasts

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Activation of pyruvate dehydrogenase by direct addition of insulin to an isolated plasma membrane/mitochondria mixture: evidence for generated of insulin's second messenger in a subcellular system

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mitochondrial Encephalomyopathy Associated with a Novel Mutation in the Mitochondrial tRNAleu(UUR)Gene (A3243T)

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Molecular basis of lipoamide dehydrogenase deficiency in Ashkenazi Jews

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mitochondrial DNA, aconitase 'wraps' it up.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

The alpha-ketoglutarate dehydrogenase complex

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Measurement of totally activated pyruvate dehydrogenase complex activity in human muscle: evaluation of a useful assay

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Controls of citrate synthase activity

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Single-gene disorders: what role could moonlighting enzymes play?

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

The single translation product of the FUM1 gene (fumarase) is processed in mitochondria before being distributed between the cytosol and mitochondria in Saccharomyces cerevisiae

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Friedreich ataxia: III. Mitochondrial malic enzyme deficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Germline mutations in FH predispose to dominantly inherited uterine fibroids, skin leiomyomata and papillary renal cell cancer

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Estimation of pyruvate dehydrogenase (E1) activity in human skeletal muscle; three cases with E1 deficiency.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Reversible transdominant inhibition of a metabolic pathway. In vivo evidence of interaction between two sequential tricarboxylic acid cycle enzymes in yeast

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Novel mutations in FH and expansion of the spectrum of phenotypes expressed in families with hereditary leiomyomatosis and renal cell cancer

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Fumaric aciduria: a new organic aciduria, associated with mental retardation and speech impairment.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Reduction of ubiquinone by lipoamide dehydrogenase

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

A decrease in glycine cleavage activity in the liver of a patient with dihydrolipoyl dehydrogenase deficiency

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

Fumarase deficiency: a new cause of mitochondrial encephalomyopathy.

1 reference

https://api.crossref.org/works/10.1016%2FS0091-679X%2806%2980010-5

7 January 2021

10.1016/S0091-679X(06)80010-5

Identifiers

DOI

1 reference

16 August 2017

1 reference

PubMed ID

17445696