Pre-clinical evaluation of cysteamine bitartrate as a therapeutic agent for mitochondrial respiratory chain disease

Mitochondrial medicine in the omics era

(2018) Joyeeta Rahman et al.   LANCET

8-year retrospective analysis of intravenous arginine therapy for acute metabolic strokes in pediatric mitochondrial disease

(2018) Rebecca D. Ganetzky et al.   MOLECULAR GENETICS AND METABOLISM

N -acetylcysteine and vitamin E rescue animal longevity and cellular oxidative stress in pre-clinical models of mitochondrial complex I disease

(2018) Erzsebet Polyak et al.   MOLECULAR GENETICS AND METABOLISM

Pharmacologic modeling of primary mitochondrial respiratory chain dysfunction in zebrafish

(2018) James Byrnes et al.   NEUROCHEMISTRY INTERNATIONAL

Mitochondrial disease patient motivations and barriers to participate in clinical trials

(2018) Zarazuela Zolkipli-Cunningham et al.   PLoS One

Mitochondrial DNA m.3243A > G heteroplasmy affects multiple aging phenotypes and risk of mortality

(2018) Gregory J. Tranah et al.   Scientific Reports

Altered mTOR signalling in nephropathic cystinosis

(2016) Ekaterina A. Ivanova et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Nutritional interventions in primary mitochondrial disorders: Developing an evidence base

(2016) Kathryn M. Camp et al.   MOLECULAR GENETICS AND METABOLISM

Using C. elegans Forward and Reverse Genetics to Identify New Compounds with Anthelmintic Activity

(2016) Mark D. Mathew et al.   PLoS Neglected Tropical Diseases

Leigh syndrome: One disorder, more than 75 monogenic causes

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

Inhibiting cytosolic translation and autophagy improves health in mitochondrial disease

(2015) Min Peng et al.   HUMAN MOLECULAR GENETICS

Whole exome sequencing of suspected mitochondrial patients in clinical practice

(2015) Saskia B. Wortmann et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Pharmacologic targeting of sirtuin and PPAR signaling improves longevity and mitochondrial physiology in respiratory chain complex I mutant Caenorhabditis elegans

(2015) Shana McCormack et al.   MITOCHONDRION

Integrated transcriptome analysis across mitochondrial disease etiologies and tissues improves understanding of common cellular adaptations to respiratory chain dysfunction

(2014) Zhe Zhang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Treatment of Mitochondrial Disorders

(2014) Gregory M. Enns   JOURNAL OF CHILD NEUROLOGY

In vivo metabolic flux profiling with stable isotopes discriminates sites and quantifies effects of mitochondrial dysfunction in C. elegans

(2014) Samantha Schrier Vergano et al.   MOLECULAR GENETICS AND METABOLISM

Supplement Timing of Cranberry Extract Plays a Key Role in Promoting Caenorhabditis elegans Healthspan

(2014) Sujay Guha et al.   Nutrients

Mutations in FBXL4, Encoding a Mitochondrial Protein, Cause Early-Onset Mitochondrial Encephalomyopathy

(2013) Xiaowu Gai et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Development of pharmacological strategies for mitochondrial disorders

(2013) M Kanabus et al.   BRITISH JOURNAL OF PHARMACOLOGY

Cysteamine Modulates Oxidative Stress and Blocks Myofibroblast Activity in CKD

(2013) D. M. Okamura et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Fiji: an open-source platform for biological-image analysis

(2012) Johannes Schindelin et al.   NATURE METHODS

Inactivation of nitric oxide by cytochrome c oxidase under steady-state oxygen conditions

(2009) David C. Unitt et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial respiratory chain dysfunction variably increases oxidant stress in Caenorhabditis elegans

(2009) Stephen Dingley et al.   MITOCHONDRION

The in-depth evaluation of suspected mitochondrial disease

(2008) Richard H. Haas et al.   MOLECULAR GENETICS AND METABOLISM

Metabolic pathway profiling of mitochondrial respiratory chain mutants in C. elegans

(2008) M.J. Falk et al.   MOLECULAR GENETICS AND METABOLISM

The role of antioxidant supplement in immune system, neoplastic, and neurodegenerative disorders: a point of view for an assessment of the risk/benefit profile

(2008) Daria Brambilla et al.   Nutrition Journal

Mitochondrial uncouplers act synergistically with the fumigant phosphine to disrupt mitochondrial membrane potential and cause cell death

(2008) Nicholas Valmas et al.   TOXICOLOGY