The Role of Oxidative Stress and Bioenergetic Dysfunction in Sulfite Oxidase Deficiency: Insights from Animal Models

Evaluation of mitochondrial bioenergetics, dynamics, endoplasmic reticulum-mitochondria crosstalk, and reactive oxygen species in fibroblasts from patients with complex I deficiency

(2018) Guilhian Leipnitz et al.   Scientific Reports

Disruption of Energy Transfer and Redox Status by Sulfite in Hippocampus, Striatum, and Cerebellum of Developing Rats

(2017) Leonardo de Moura Alvorcem et al.   NEUROTOXICITY RESEARCH

AMPK Maintains Cellular Metabolic Homeostasis through Regulation of Mitochondrial Reactive Oxygen Species

(2017) Rebecca C. Rabinovitch et al.   Cell Reports

Mechanistic basis of hypermethioninemia

(2016) Bruna M. Schweinberger et al.   AMINO ACIDS

Higher susceptibility of cerebral cortex and striatum to sulfite neurotoxicity in sulfite oxidase-deficient rats

(2016) Mateus Grings et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Molybdenum cofactor and human disease

(2016) Guenter Schwarz   CURRENT OPINION IN CHEMICAL BIOLOGY

Molybdenum cofactor and isolated sulphite oxidase deficiencies: Clinical and molecular spectrum among Egyptian patients

(2016) Maha S. Zaki et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency

(2016) Mateus Struecker da Rosa et al.   FREE RADICAL RESEARCH

1,25-Dihydroxyvitamin D3 exerts neuroprotective effects in an ex vivo model of mild hyperhomocysteinemia

(2016) Aline Longoni et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Mitochondrial Ca2+ and regulation of the permeability transition pore

(2016) Stephen Hurst et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

The rise of mitochondria in medicine

(2016) Martin Picard et al.   MITOCHONDRION

Hypoxanthine Intrastriatal Administration Alters Neuroinflammatory Profile and Redox Status in Striatum of Infant and Young Adult Rats

(2016) Helena Biasibetti et al.   MOLECULAR NEUROBIOLOGY

Sulfite triggers sustained calcium overload in cultured cortical neurons via a redox-dependent mechanism

(2016) Xiao Wang et al.   TOXICOLOGY LETTERS

Calcium and mitochondria in the regulation of cell death

(2015) Sten Orrenius et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

New insights into PGC-1 coactivators: redefining their role in the regulation of mitochondrial function and beyond

(2015) Josep A. Villena   FEBS Journal

In vitro evidence that sulfite impairs glutamatergic neurotransmission and inhibits glutathione metabolism-related enzymes in rat cerebral cortex

(2015) Belisa Parmeggiani et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Efficacy and safety of cyclic pyranopterin monophosphate substitution in severe molybdenum cofactor deficiency type A: a prospective cohort study

(2015) Bernd C Schwahn et al.   LANCET

Intracerebral Glycine Administration Impairs Energy and Redox Homeostasis and Induces Glial Reactivity in Cerebral Cortex of Newborn Rats

(2015) Alana Pimentel Moura et al.   MOLECULAR NEUROBIOLOGY

Severe Hyperhomocysteinemia Decreases Respiratory Enzyme and Na+-K+ ATPase Activities, and Leads to Mitochondrial Alterations in Rat Amygdala

(2015) Janaína Kolling et al.   NEUROTOXICITY RESEARCH

Sulfite disrupts brain mitochondrial energy homeostasis and induces mitochondrial permeability transition pore opening via thiol group modification

(2014) Mateus Grings et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Sulfite-oxidizing enzymes

(2014) Ulrike Kappler et al.   JOURNAL OF BIOLOGICAL INORGANIC CHEMISTRY

Effect of physical exercise on changes in activities of creatine kinase, cytochrome c oxidase and ATP levels caused by ovariectomy

(2014) Cassiana Siebert et al.   METABOLIC BRAIN DISEASE

Prenatal Multicystic Encephalopathy in Isolated Sulfite Oxidase Deficiency With a Novel Mutaion

(2014) Li-Wen Chen et al.   PEDIATRIC NEUROLOGY

The effect of ingested sulfite on visual evoked potentials, lipid peroxidation, and antioxidant status of brain in normal and sulfite oxidase-deficient aged rats

(2014) Ozlem Ozsoy et al.   TOXICOLOGY AND INDUSTRIAL HEALTH

Infantile Spasms and Hyperekplexia Associated With Isolated Sulfite Oxidase Deficiency

(2014) J. Lloyd Holder et al.   JAMA Neurology

Determination of sulfite with emphasis on biosensing methods: a review

(2013) Chandra Shekhar Pundir et al.   ANALYTICAL AND BIOANALYTICAL CHEMISTRY

Sulfite oxidase deficiency – An unusual late and mild presentation

(2013) Susana Rocha et al.   BRAIN & DEVELOPMENT

Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models

(2013) J C Komen et al.   BRITISH JOURNAL OF PHARMACOLOGY

Disturbance of brain energy and redox homeostasis provoked by sulfite and thiosulfate: Potential pathomechanisms involved in the neuropathology of sulfite oxidase deficiency

(2013) Mateus Grings et al.   GENE

Glycine Intracerebroventricular Administration Disrupts Mitochondrial Energy Homeostasis in Cerebral Cortex and Striatum of Young Rats

(2013) Alana Pimentel Moura et al.   NEUROTOXICITY RESEARCH

Early Features in Neuroimaging of Two Siblings With Molybdenum Cofactor Deficiency

(2013) Ryuzo Higuchi et al.   PEDIATRICS

Human Sulfide:Quinone Oxidoreductase Catalyzes the First Step in Hydrogen Sulfide Metabolism and Produces a Sulfane Sulfur Metabolite

(2012) Michael R. Jackson et al.   BIOCHEMISTRY

Molybdenum cofactor deficiency: Review of 12 cases (MoCD and review)

(2012) Erhan Bayram et al.   EUROPEAN JOURNAL OF PAEDIATRIC NEUROLOGY

Merit of quinacrine in the decrease of ingested sulfite-induced toxic action in rat brain

(2012) Ceren Kencebay et al.   FOOD AND CHEMICAL TOXICOLOGY

Regulation of PGC-1α, a nodal regulator of mitochondrial biogenesis

(2011) Pablo J Fernandez-Marcos et al.   AMERICAN JOURNAL OF CLINICAL NUTRITION

Species Differences in the Effects of Bezafibrate as a Potential Treatment of Mitochondrial Disorders

(2011) Fatima Djouadi et al.   Cell Metabolism

Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease

(2011) Ashu Johri et al.   HUMAN MOLECULAR GENETICS

Mitochondrial aconitase knockdown attenuates paraquat-induced dopaminergic cell death via decreased cellular metabolism and release of iron and H2O2

(2011) David Cantu et al.   JOURNAL OF NEUROCHEMISTRY

Behavioral and neurochemical effects of proline

(2011) Angela T. S. Wyse et al.   METABOLIC BRAIN DISEASE

Clinical Neuroimaging Features and Outcome in Molybdenum Cofactor Deficiency

(2011) Kayal Vijayakumar et al.   PEDIATRIC NEUROLOGY

Neonatal Astrocyte Damage Is Sufficient to Trigger Progressive Striatal Degeneration in a Rat Model of Glutaric Acidemia-I

(2011) Silvia Olivera-Bravo et al.   PLoS One

Long-Term Follow-Up of Bezafibrate Treatment in Patients With the Myopathic Form of Carnitine Palmitoyltransferase 2 Deficiency

(2010) J P Bonnefont et al.   CLINICAL PHARMACOLOGY & THERAPEUTICS

The pro-oxidant chromium(VI) inhibits mitochondrial complex I, complex II, and aconitase in the bronchial epithelium: EPR markers for Fe–S proteins

(2010) Charles R. Myers et al.   FREE RADICAL BIOLOGY AND MEDICINE

Molybdenum cofactor deficiency: Mutations in GPHN, MOCS1, and MOCS2

(2010) Jochen Reiss et al.   HUMAN MUTATION

Mitochondrial dysfunction and oxidative stress: a contributing link to acquired epilepsy?

(2010) Simon Waldbaum et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Effect of sulfite on antioxidant enzymes and lipid peroxidation in normal and sulfite oxidase-deficient rat erythrocytes

(2010) Oktay Hasan Ozturk et al.   JOURNAL OF PHYSIOLOGY AND BIOCHEMISTRY

Successful Treatment of Molybdenum Cofactor Deficiency Type A With cPMP

(2010) A. Veldman et al.   PEDIATRICS

Mitochondrial and apoptotic neuronal death signaling pathways in cerebral ischemia

(2009) Kuniyasu Niizuma et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Functional deficiencies of sulfite oxidase: Differential diagnoses in neonates presenting with intractable seizures and cystic encephalomalacia

(2009) Jörn Oliver Sass et al.   BRAIN & DEVELOPMENT

Clinical effects of sulphite additives

(2009) H. Vally et al.   CLINICAL AND EXPERIMENTAL ALLERGY

Oxidative risk for atherothrombotic cardiovascular disease

(2009) Jane A. Leopold et al.   FREE RADICAL BIOLOGY AND MEDICINE

Oxidative Stress and Its Role in the Pathogenesis of Ischaemic Stroke

(2009) C. L. Allen et al.   International Journal of Stroke

Molybdenum cofactors, enzymes and pathways

(2009) Günter Schwarz et al.   NATURE

S100B's double life: Intracellular regulator and extracellular signal

(2008) Rosario Donato et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Transcriptional control of mitochondrial biogenesis: the central role of PGC-1 

(2008) R. Ventura-Clapier et al.   CARDIOVASCULAR RESEARCH

Effect of sulfite treatment on total antioxidant capacity, total oxidant status, lipid hydroperoxide, and total free sulfydryl groups contents in normal and sulfite oxidase-deficient rat plasma

(2008) Emine Nur Herken et al.   CELL BIOLOGY AND TOXICOLOGY

Mechanism of sulfite cytotoxicity in isolated rat hepatocytes

(2008) Hossein Niknahad et al.   CHEMICO-BIOLOGICAL INTERACTIONS

Effect of alpha-lipoic acid on visual evoked potentials in rats exposed to sulfite

(2008) Narin Derin et al.   NEUROTOXICOLOGY AND TERATOLOGY

Dysfunctional astrocytes as key players in the pathogenesis of central nervous system disorders

(2007) Jacques De Keyser et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES