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

Phosphorylation of voltage-dependent anion channel by c-Jun N-terminal Kinase-3 leads to closure of the channel

(2015) Rajeev Gupta et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

Glial Lipid Droplets and ROS Induced by Mitochondrial Defects Promote Neurodegeneration

(2015) Lucy Liu et al.   CELL

Evidence for a common biological pathway linking three Parkinson's disease-causing genes:parkin,PINK1andDJ-1

(2015) Celia van der Merwe et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Markov chain Monte Carlo based analysis of post-translationally modified VDAC gating kinetics

(2015) Shivendra G. Tewari et al.   Frontiers in Physiology

Hereditary Leiomyomatosis and Renal Cell Carcinoma Syndrome–associated Renal Cancer

(2014) Ying-Bei Chen et al.   AMERICAN JOURNAL OF SURGICAL PATHOLOGY

Identification of protein succination as a novel modification of tubulin

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

Influence of Protein – Micelle Ratios and Cysteine Residues on the Kinetic Stability and Unfolding Rates of Human Mitochondrial VDAC-2

(2014) Svetlana Rajkumar Maurya et al.   PLoS One

A Selected Core Microbiome Drives the Early Stages of Three Popular Italian Cheese Manufactures

(2014) Francesca De Filippis et al.   PLoS One

ProteinS-glutathionylation: from current basics to targeted modifications

(2014) Doina Popov   ARCHIVES OF PHYSIOLOGY AND BIOCHEMISTRY

Oxidative damage to macromolecules in human Parkinson disease and the rotenone model

(2013) Laurie H. Sanders et al.   FREE RADICAL BIOLOGY AND MEDICINE

Modulation of Human Mitochondrial Voltage-dependent Anion Channel 2 (hVDAC-2) Structural Stability by Cysteine-assisted Barrel-lipid Interactions

(2013) Svetlana Rajkumar Maurya et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The succinated proteome

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

Glutathione: A redox signature in monitoring EPI-743 therapy in children with mitochondrial encephalomyopathies

(2013) Anna Pastore et al.   MOLECULAR GENETICS AND METABOLISM

Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

(2013) Nicola Ternette et al.   Cell Reports

Mitochondrial stress causes increased succination of proteins in adipocytes in response to glucotoxicity

(2012) Norma Frizzell et al.   BIOCHEMICAL JOURNAL

Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts

(2012) Federica Valsecchi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Fatal breathing dysfunction in a mouse model of Leigh syndrome

(2012) Albert Quintana et al.   JOURNAL OF CLINICAL INVESTIGATION

Proteomic Analysis of Covalent Modifications of Tubulins by Isothiocyanates

(2012) Zhen Xiao et al.   JOURNAL OF NUTRITION

Primary fibroblasts of NDUFS4−/− mice display increased ROS levels and aberrant mitochondrial morphology

(2012) Federica Valsecchi et al.   MITOCHONDRION

Mouse mtDNA mutant model of Leber hereditary optic neuropathy

(2012) C. S. Lin et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

4-Hydroxy-2-Nonenal, a Reactive Product of Lipid Peroxidation, and Neurodegenerative Diseases: A Toxic Combination Illuminated by Redox Proteomics Studies

(2011) Marzia Perluigi et al.   ANTIOXIDANTS & REDOX SIGNALING

Understanding mitochondrial complex I assembly in health and disease

(2011) Masakazu Mimaki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

VDAC inhibition by tubulin and its physiological implications

(2011) Tatiana K. Rostovtseva et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Renal Cyst Formation in Fh1-Deficient Mice Is Independent of the Hif/Phd Pathway: Roles for Fumarate in KEAP1 Succination and Nrf2 Signaling

(2011) Julie Adam et al.   CANCER CELL

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

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

Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson’s disease model

(2011) Won-Seok Choi et al.   JOURNAL OF CELL BIOLOGY

Aberrant succination of proteins in fumarate hydratase-deficient mice and HLRCC patients is a robust biomarker of mutation status

(2011) Chiara Bardella et al.   JOURNAL OF PATHOLOGY

Mitochondrial complex I deficiency of nuclear origin

(2011) Hélène Pagniez-Mammeri et al.   MOLECULAR GENETICS AND METABOLISM

Mitochondrial complex I deficiency of nuclear origin

(2011) Hélène Pagniez-Mammeri et al.   MOLECULAR GENETICS AND METABOLISM

Tissue Distribution of S-(2-Succino)cysteine (2SC), a Biomarker of Mitochondrial Stress in Obesity and Diabetes

(2011) Sonia A. Thomas et al.   Obesity

VDAC1 cysteine residues: topology and function in channel activity and apoptosis

(2010) Lior Aram et al.   BIOCHEMICAL JOURNAL

Involvements of the lipid peroxidation product, HNE, in the pathogenesis and progression of Alzheimer's disease☆

(2010) D. Allan Butterfield et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

A meta-analysis of two-dimensional electrophoresis pattern of the Parkinson's disease-related protein DJ-1

(2010) Massimo Natale et al.   BIOINFORMATICS

Recent Advances in the Genetics of Mitochondrial Encephalopathies

(2010) Elena J. Tucker et al.   Current Neurology and Neuroscience Reports

Inhibition of Bak Activation by VDAC2 Is Dependent on the Bak Transmembrane Anchor

(2010) Michael Lazarou et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Acyl-biotinyl Exchange Chemistry and Mass Spectrometry-Based Analysis of Palmitoylation Sites of In Vitro Palmitoylated Rat Brain Tubulin

(2010) Zhiqiang Zhao et al.   PROTEIN JOURNAL

Pathogenetic mechanisms in hereditary dysfunctions of complex I of the respiratory chain in neurological diseases

(2009) Sergio Papa et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Succination of Thiol Groups in Adipose Tissue Proteins in Diabetes

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

NDUFS4 mutations cause Leigh syndrome with predominant brainstem involvement

(2009) E. Leshinsky-Silver et al.   MOLECULAR GENETICS AND METABOLISM

Vestibular System: The Many Facets of a Multimodal Sense

(2008) Dora E. Angelaki et al.   Annual Review of Neuroscience

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

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

Glycogen Synthase Kinase 3 Inhibition Slows Mitochondrial Adenine Nucleotide Transport and Regulates Voltage-Dependent Anion Channel Phosphorylation

(2008) Samarjit Das et al.   CIRCULATION RESEARCH

Reactive oxygen species act remotely to cause synapse loss in a Drosophila model of developmental mitochondrial encephalopathy

(2008) J. D. Mast et al.   DEVELOPMENT

Leigh and Leigh-Like Syndrome in Children and Adults

(2008) Josef Finsterer   PEDIATRIC NEUROLOGY

Mitochondrial complex I inhibition is not required for dopaminergic neuron death induced by rotenone, MPP+, or paraquat

(2008) W.-S. Choi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA