Defective function of α-ketoglutarate dehydrogenase exacerbates mitochondrial ATP deficits during complex I deficiency

Ultrasensitive HPLC-MS Quantification of S-(2-Succino) Cysteine Based on Ethanol/Acetyl Chloride Derivatization in Fumarate Accumulation Cells

(2023) Ying Liu et al.   Analytical Chemistry

Iron status influences mitochondrial disease progression in Complex I-deficient mice

(2023) CJ Kelly et al.   eLife

DLST mutations in pheochromocytoma and paraganglioma cause proteome hyposuccinylation and metabolic remodeling

(2023) Sara Mellid et al.   Cancer Communications

Altered succinylation of mitochondrial proteins, APP and tau in Alzheimer’s disease

(2022) Yun Yang et al.   Nature Communications

Aberrant and glutamate metabolism linked to regional neurodegeneration in a mouse model of Leigh syndrome

(2021) Karin Terburgh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Circulating markers of NADH-reductive stress correlate with mitochondrial disease severity

(2021) Rohit Sharma et al.   JOURNAL OF CLINICAL INVESTIGATION

Structure of the dihydrolipoamide succinyltransferase (E2) component of the human alpha-ketoglutarate dehydrogenase complex (hKGDHc) revealed by cryo-EM and cross-linking mass spectrometry: Implications for the overall hKGDHc structure

(2021) Balint Nagy et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Cross-comparison of systemic and tissue-specific metabolomes in a mouse model of Leigh syndrome

(2021) Karin Terburgh et al.   Metabolomics

Exclusive neuronal detection of KGDHC-specific subunits in the adult human brain cortex despite pancellular protein lysine succinylation

(2020) Arpad Dobolyi et al.   Brain Structure & Function

An Oncometabolite Isomer Rapidly Induces a Pathophysiological Protein Modification

(2020) Sarah E. Bergholtz et al.   ACS Chemical Biology

Scalable molecular dynamics on CPU and GPU architectures with NAMD

(2020) James C. Phillips et al.   JOURNAL OF CHEMICAL PHYSICS

A biallelic pathogenic variant in the OGDH gene results in a neurological disorder with features of a mitochondrial disease

(2020) Zheng Yie Yap et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Regional metabolic signatures in the Ndufs4(KO) mouse brain implicate defective glutamate/α-ketoglutarate metabolism in mitochondrial disease

(2020) Simon C. Johnson et al.   MOLECULAR GENETICS AND METABOLISM

ABHD11 maintains 2-oxoglutarate metabolism by preserving functional lipoylation of the 2-oxoglutarate dehydrogenase complex

(2020) Peter S. J. Bailey et al.   Nature Communications

Mitochondrial Proteome of Affected Glutamatergic Neurons in a Mouse Model of Leigh Syndrome

(2020) Alejandro Gella et al.   Frontiers in Cell and Developmental Biology

SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease

(2020) Philipp Gut et al.   Nature Communications

Targeting NAD+ Metabolism as Interventions for Mitochondrial Disease

(2019) Chi Fung Lee et al.   Scientific Reports

Succinylation Links Metabolism to Protein Functions

(2019) Yun Yang et al.   NEUROCHEMICAL RESEARCH

A chemoproteomic portrait of the oncometabolite fumarate

(2019) Rhushikesh A. Kulkarni et al.   Nature Chemical Biology

Recurrent Germline DLST Mutations in Individuals with Multiple Pheochromocytomas and Paragangliomas

(2019) Laura Remacha et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Defined neuronal populations drive fatal phenotype in a mouse model of Leigh syndrome

(2019) Irene Bolea et al.   eLife

Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation

(2019) Isha H. Jain et al.   Cell Metabolism

Fumarate and oxidative stress synergize to promote stability of C/EBP homologous protein in the adipocyte

(2019) Allison M. Manuel et al.   FREE RADICAL BIOLOGY AND MEDICINE

LIPT1 deficiency presenting as early infantile epileptic encephalopathy, Leigh disease, and secondary pyruvate dehydrogenase complex deficiency

(2018) Robert C. Stowe et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species

(2018) Natalia S. Nemeria et al.   FREE RADICAL BIOLOGY AND MEDICINE

Metabolomics of Ndufs4−/− skeletal muscle: Adaptive mechanisms converge at the ubiquinone-cycle

(2018) Karin Terburgh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Identification of Novel Protein Targets of Dimethyl Fumarate Modification in Neurons and Astrocytes Reveals Actions Independent of Nrf2 Stabilization

(2018) Gerardo G. Piroli et al.   MOLECULAR & CELLULAR PROTEOMICS

Mild metabolic perturbations alter succinylation of mitochondrial proteins

(2017) Huanlian Chen et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Methylene blue stimulates substrate-level phosphorylation catalysed by succinyl–CoA ligase in the citric acid cycle

(2017) T. Komlódi et al.   NEUROPHARMACOLOGY

Hypoxia treatment reverses neurodegenerative disease in a mouse model of Leigh syndrome

(2017) Michele Ferrari et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease

(2017) Ria de Haas et al.   Scientific Reports

Acidic pH Is a Metabolic Switch for 2-Hydroxyglutarate Generation and Signaling

(2016) Sergiy M. Nadtochiy et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ndufs4 related Leigh syndrome: A case report and review of the literature

(2016) Juan Darío Ortigoza-Escobar et al.   MITOCHONDRION

CHARMM36m: an improved force field for folded and intrinsically disordered proteins

(2016) Jing Huang et al.   NATURE METHODS

Mild mitochondrial metabolic deficits by α-ketoglutarate dehydrogenase inhibition cause prominent changes in intracellular autophagic signaling: Potential role in the pathobiology of Alzheimer's disease

(2016) Kalpita Banerjee et al.   NEUROCHEMISTRY INTERNATIONAL

Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain

(2016) Ernst-Bernhard Kayser et al.   PLoS One

Complementation of mitochondrial electron transport chain by manipulation of the NAD+/NADH ratio

(2016) D. V. Titov et al.   SCIENCE

Hypoxia as a therapy for mitochondrial disease

(2016) I. H. Jain et al.   SCIENCE

Differential Aspartate Usage Identifies a Subset of Cancer Cells Particularly Dependent on OGDH

(2016) Eric L. Allen et al.   Cell Reports

Leigh syndrome: One disorder, more than 75 monogenic causes

(2015) Nicole J. Lake et al.   ANNALS OF NEUROLOGY

Skeletal muscle mitochondria of NDUFS4−/− mice display normal maximal pyruvate oxidation and ATP production

(2015) Mohammad T. Alam et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

The effect of glucose concentration and sodium phenylbutyrate treatment on mitochondrial bioenergetics and ER stress in 3T3-L1 adipocytes

(2015) Ross M. Tanis et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Increased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia

(2015) Ganesh R. Manjeri et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Alpha-ketoglutarate dehydrogenase complex-dependent succinylation of proteins in neurons and neuronal cell lines

(2015) Gary E. Gibson et al.   JOURNAL OF NEUROCHEMISTRY

Succination is Increased on Select Proteins in the Brainstem of the NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) Knockout Mouse, a Model of Leigh Syndrome

(2015) Gerardo G. Piroli et al.   MOLECULAR & CELLULAR PROTEOMICS

Identification of protein succination as a novel modification of tubulin

(2014) Gerardo G. Piroli et al.   BIOCHEMICAL JOURNAL

Neuronal and astrocyte dysfunction diverges from embryonic fibroblasts in theNdufs4fky/fkymouse

(2014) Matthew J. Bird et al.   BIOSCIENCE REPORTS

Proteomic Analysis of Gliosomes from Mouse Brain: Identification and Investigation of Glial Membrane Proteins

(2014) Karen E. Carney et al.   JOURNAL OF PROTEOME RESEARCH

The negative impact of α-ketoglutarate dehydrogenase complex deficiency on matrix substrate-level phosphorylation

(2013) Gergely Kiss et al.   FASEB JOURNAL

The succinated proteome

(2013) Eric D. Merkley et al.   MASS SPECTROMETRY REVIEWS

SIRT5-Mediated Lysine Desuccinylation Impacts Diverse Metabolic Pathways

(2013) Jeongsoon Park et al.   MOLECULAR CELL

Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

(2013) Nicola Ternette et al.   Cell Reports

Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity

(2011) M. A. Calvaruso et al.   HUMAN MOLECULAR GENETICS

Mutations in the Dimer Interface of Dihydrolipoamide Dehydrogenase Promote Site-specific Oxidative Damages in Yeast and Human Cells

(2011) Rachael A. Vaubel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Identification of lysine succinylation as a new post-translational modification

(2010) Zhihong Zhang et al.   Nature Chemical Biology

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Succination of Thiol Groups in Adipose Tissue Proteins in Diabetes

(2009) Norma Frizzell et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inhibition of the alpha-ketoglutarate dehydrogenase-mediated reactive oxygen species generation by lipoic acid

(2009) Attila Ambrus et al.   JOURNAL OF NEUROCHEMISTRY

Mice deficient in dihydrolipoyl succinyl transferase show increased vulnerability to mitochondrial toxins

(2009) Lichuan Yang et al.   NEUROBIOLOGY OF DISEASE

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism