Impaired TCA cycle flux in mitochondria in skeletal muscle from type 2 diabetic subjects: Marker or maker of the diabetic phenotype?

Familial pheochromocytoma

(2014) Zoran Erlic et al.   Hormones-International Journal of Endocrinology and Metabolism

The dynamic equilibrium between ATP synthesis and ATP consumption is lower in isolated mitochondria from myotubes established from type 2 diabetic subjects compared to lean control

(2011) Ariane D. Minet et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

SDH mutations in cancer

(2011) Chiara Bardella et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The interplay between SUCLA2, SUCLG2, and mitochondrial DNA depletion

(2011) Chaya Miller et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

NF-Y and the transcriptional activation of CCAAT promoters

(2011) Diletta Dolfini et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Isocitrate dehydrogenase mutations in gliomas

(2011) Changcun Guo et al.   CURRENT OPINION IN NEUROLOGY

Hydrogen Peroxide Production Is Not Primarily Increased in Human Myotubes Established from Type 2 Diabetic Subjects

(2011) A. D. Minet et al.   JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM

Up-regulation of the mitochondrial malate dehydrogenase by oxidative stress is mediated by miR-743a

(2011) Qingli Shi et al.   JOURNAL OF NEUROCHEMISTRY

IDH1 and IDH2 mutations are frequent events in central chondrosarcoma and central and periosteal chondromas but not in other mesenchymal tumours

(2011) M Fernanda Amary et al.   JOURNAL OF PATHOLOGY

Succinate Dehydrogenase Is a Direct Target of Sirtuin 3 Deacetylase Activity

(2011) Lydia W. S. Finley et al.   PLoS One

Profiling the effects of isocitrate dehydrogenase 1 and 2 mutations on the cellular metabolome

(2011) Z. J. Reitman et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Revisiting the TCA cycle: signaling to tumor formation

(2011) Nuno Raimundo et al.   TRENDS IN MOLECULAR MEDICINE

Mitochondrial proteome analysis reveals altered expression of voltage dependent anion channels in pancreatic β-cells exposed to high glucose

(2011) Meftun Ahmed et al.   Islets

Nuclear Control of Respiratory Chain Expression by Nuclear Respiratory Factors and PGC-1-Related Coactivator

(2010) Richard C. Scarpulla   Annals of the New York Academy of Sciences

ATP synthesis is impaired in isolated mitochondria from myotubes established from type 2 diabetic subjects

(2010) Ariane D. Minet et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Mitochondrial mass is inversely correlated to complete lipid oxidation in human myotubes

(2010) Michael Gaster   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

IDH1 mutations are detected in 6.6% of 1414 AML patients and are associated with intermediate risk karyotype and unfavorable prognosis in adults younger than 60 years and unmutated NPM1 status

(2010) S. Schnittger et al.   BLOOD

Disease-specific autoantibodies in primary biliary cirrhosis

(2010) Dimitrios P. Bogdanos et al.   CLINICA CHIMICA ACTA

Warburg tumours and the mechanisms of mitochondrial tumour suppressor genes. Barking up the right tree?

(2010) Jean-Pierre Bayley et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase

(2010) Aviva Levitas et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

SDHA is a tumor suppressor gene causing paraganglioma

(2010) Nelly Burnichon et al.   HUMAN MOLECULAR GENETICS

Each Conserved Active Site Tyr in the Three Subunits of Human Isocitrate Dehydrogenase Has a Different Function

(2010) Mayura Dange et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The severity of phenotype linked to SUCLG1 mutations could be correlated with residual amount of SUCLG1 protein

(2010) C. Rouzier et al.   JOURNAL OF MEDICAL GENETICS

The Profile of Mitochondrial Proteins and Their Phosphorylation Signaling Network in INS-1 β Cells

(2010) Ziyou Cui et al.   JOURNAL OF PROTEOME RESEARCH

Marked mitochondrial DNA depletion associated with a novel SUCLG1 gene mutation resulting in lethal neonatal acidosis, multi-organ failure, and interrupted aortic arch

(2010) Henry Rivera et al.   MITOCHONDRION

Phosphoproteome Analysis of Functional Mitochondria Isolated from Resting Human Muscle Reveals Extensive Phosphorylation of Inner Membrane Protein Complexes and Enzymes

(2010) Xiaolu Zhao et al.   MOLECULAR & CELLULAR PROTEOMICS

Phosphoproteome Analysis Reveals Regulatory Sites in Major Pathways of Cardiac Mitochondria

(2010) Ning Deng et al.   MOLECULAR & CELLULAR PROTEOMICS

Erratum: Cancer-associated IDH1 mutations produce 2-hydroxyglutarate

(2010) Lenny Dang et al.   NATURE

p53 downregulates the gene expression of mitochondrial aconitase in human prostate carcinoma cells

(2010) Ke-Hung Tsui et al.   PROSTATE

IDH2 Mutations in Patients with D-2-Hydroxyglutaric Aciduria

(2010) M. Kranendijk et al.   SCIENCE

Regulation of Cellular Metabolism by Protein Lysine Acetylation

(2010) S. Zhao et al.   SCIENCE

Fumarase: A Mitochondrial Metabolic Enzyme and a Cytosolic/Nuclear Component of the DNA Damage Response

(2010) Ohad Yogev et al.   PLOS BIOLOGY

Reduced lipid oxidation in myotubes established from obese and type 2 diabetic subjects

(2009) Michael Gaster   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Reduced TCA flux in diabetic myotubes: A governing influence on the diabetic phenotype?

(2009) Michael Gaster   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Succinyl-CoA Synthetase Is a Phosphate Target for the Activation of Mitochondrial Metabolism

(2009) Darci Phillips et al.   BIOCHEMISTRY

Regulation of Succinate Dehydrogenase Activity by SIRT3 in Mammalian Mitochondria

(2009) Huseyin Cimen et al.   BIOCHEMISTRY

Regulation of mitochondrial dehydrogenases by calcium ions

(2009) Richard M. Denton   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

A nucleocytoplasmic malate dehydrogenase regulates p53 transcriptional activity in response to metabolic stress

(2009) S M Lee et al.   CELL DEATH AND DIFFERENTIATION

Regulation of mitochondrial aconitase by phosphorylation in diabetic rat heart

(2009) G. Lin et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Mitochondrial reactive oxygen species generation in obese non-diabetic and type 2 diabetic participants

(2009) M. A. Abdul-Ghani et al.   DIABETOLOGIA

Contrasting clinical manifestations of SDHB and VHL associated chromaffin tumours

(2009) Umasuthan Srirangalingam et al.   ENDOCRINE-RELATED CANCER

A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria

(2009) Elsebet Ostergaard et al.   EUROPEAN JOURNAL OF PEDIATRICS

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins

(2009) Blanche Guillon et al.   FEBS Journal

Mitochondrial dihydrolipoyl succinyltransferase deficiency accelerates amyloid pathology and memory deficit in a transgenic mouse model of amyloid deposition

(2009) Magali Dumont et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mitochondrial reticulum network dynamics in relation to oxidative stress, redox regulation, and hypoxia

(2009) Petr Ježek et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Identification and characterization of the mitochondrial targeting sequence and mechanism in human citrate synthase

(2009) Tsung-Lin Cheng et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Oxidative modification of citrate synthase by peroxyl radicals and protection with novel antioxidants

(2009) Nikolai L. Chepelev et al.   JOURNAL OF ENZYME INHIBITION AND MEDICINAL CHEMISTRY

Use of32P To Study Dynamics of the Mitochondrial Phosphoproteome

(2009) Angel M. Aponte et al.   JOURNAL OF PROTEOME RESEARCH

Dystonia and deafness due to SUCLA2 defect; Clinical course and biochemical markers in 16 children

(2009) Eva Morava et al.   MITOCHONDRION

Fumarate Hydratase Deficiency in Renal Cancer Induces Glycolytic Addiction and Hypoxia-Inducible Transcription Factor 1  Stabilization by Glucose-Dependent Generation of Reactive Oxygen Species

(2009) S. Sudarshan et al.   MOLECULAR AND CELLULAR BIOLOGY

Cancer-associated IDH1 mutations produce 2-hydroxyglutarate

(2009) Lenny Dang et al.   NATURE

SDHAF1, encoding a LYR complex-II specific assembly factor, is mutated in SDH-defective infantile leukoencephalopathy

(2009) Daniele Ghezzi et al.   NATURE GENETICS

IDH1 mutations in low-grade astrocytomas predict survival but not response to temozolomide

(2009) H. J. Dubbink et al.   NEUROLOGY

Recurring Mutations Found by Sequencing an Acute Myeloid Leukemia Genome

(2009) Elaine R. Mardis et al.   NEW ENGLAND JOURNAL OF MEDICINE

IDH1andIDH2Mutations in Gliomas

(2009) Hai Yan et al.   NEW ENGLAND JOURNAL OF MEDICINE

Obesity, diets, and social inequalities

(2009) Adam Drewnowski   NUTRITION REVIEWS

Downregulation of SRF–FOS–JUNB pathway in fumarate hydratase deficiency and in uterine leiomyomas

(2009) N Raimundo et al.   ONCOGENE

Regulation of succinate-ubiquinone reductase and fumarate reductase activities in human complex II by phosphorylation of its flavoprotein subunit

(2009) Eriko TOMITSUKA et al.   PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES

SDH5, a Gene Required for Flavination of Succinate Dehydrogenase, Is Mutated in Paraganglioma

(2009) H.-X. Hao et al.   SCIENCE

Lysine Acetylation Targets Protein Complexes and Co-Regulates Major Cellular Functions

(2009) C. Choudhary et al.   SCIENCE

Glioma-Derived Mutations in IDH1 Dominantly Inhibit IDH1 Catalytic Activity and Induce HIF-1 

(2009) S. Zhao et al.   SCIENCE

Differential metabolic consequences of fumarate hydratase and respiratory chain defects

(2008) Nuno Raimundo et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Phosphoproteome analysis of isoflurane-protected heart mitochondria: phosphorylation of adenine nucleotide translocator-1 on Tyr194 regulates mitochondrial function

(2008) Jianhua Feng et al.   CARDIOVASCULAR RESEARCH

The functional duality of iron regulatory protein 1

(2008) Karl Volz   CURRENT OPINION IN STRUCTURAL BIOLOGY

Fatty Acid Incubation of Myotubes From Humans With Type 2 Diabetes Leads to Enhanced Release of  -Oxidation Products Because of Impaired Fatty Acid Oxidation: Effects of Tetradecylthioacetic Acid and Eicosapentaenoic Acid

(2008) A. J. Wensaas et al.   DIABETES

Transcriptional profiling of myotubes from patients with type 2 diabetes: no evidence for a primary defect in oxidative phosphorylation genes

(2008) C. M. Frederiksen et al.   DIABETOLOGIA

Reduced tricarboxylic acid cycle flux in type 2 diabetes mellitus?

(2008) P. Schrauwen et al.   DIABETOLOGIA

Allosteric Motions in Structures of Yeast NAD+-specific Isocitrate Dehydrogenase

(2008) Alexander B. Taylor et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Transcriptional Regulation of SDHa Flavoprotein by Nuclear Respiratory Factor-1 Prevents Pseudo-hypoxia in Aerobic Cardiac Cells

(2008) Claude A. Piantadosi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The mitochondrial targeting sequence tilts the balance between mitochondrial and cytosolic dual localization

(2008) N. Regev-Rudzki et al.   JOURNAL OF CELL SCIENCE

Substrates and Regulation Mechanisms for the Human Mitochondrial Sirtuins Sirt3 and Sirt5

(2008) Christine Schlicker et al.   JOURNAL OF MOLECULAR BIOLOGY

Lysine Acetylation: Codified Crosstalk with Other Posttranslational Modifications

(2008) Xiang-Jiao Yang et al.   MOLECULAR CELL

Insights from retinitis pigmentosa into the roles of isocitrate dehydrogenases in the Krebs cycle

(2008) Dyonne T Hartong et al.   NATURE GENETICS

Altered expression of12S/MT-RNR1,MT-CO2/COX2, andMT-ATP6mitochondrial genes in prostate cancer

(2008) Jesús Abril et al.   PROSTATE

An Integrated Genomic Analysis of Human Glioblastoma Multiforme

(2008) D. W. Parsons et al.   SCIENCE

Reversible Inhibition of α-Ketoglutarate Dehydrogenase by Hydrogen Peroxide:  Glutathionylation and Protection of Lipoic Acid†

(2007) Milana A. B. Applegate et al.   BIOCHEMISTRY