N-acetylcysteine amide ameliorates mitochondrial dysfunction and reduces oxidative stress in hiPSC-derived dopaminergic neurons with POLG mutation

Addressing variability in iPSC-derived models of human disease: guidelines to promote reproducibility

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Mitophagy inhibits amyloid-β and tau pathology and reverses cognitive deficits in models of Alzheimer’s disease

(2019) Evandro F. Fang et al.   NATURE NEUROSCIENCE

Mitochondria in the maintenance of hematopoietic stem cells new perspectives and opportunities

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Neuroprotective effect of N-acetylcysteine against cisplatin-induced toxicity in rat brain by modulation of oxidative stress and inflammation

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Cellular alterations identified in pluripotent stem cell-derived midbrain spheroids generated from a female patient with progressive external ophthalmoplegia and parkinsonism who carries a novel variation (p.Q811R) in the POLG1 gene

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Phenotypic Reprogramming of Striatal Neurons into Dopaminergic Neuron-like Cells in the Adult Mouse Brain

(2018) Wenze Niu et al.   Stem Cell Reports

The clinical spectrum and natural history of early-onset diseases due to DNA polymerase gamma mutations

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Understanding the Epilepsy in POLG Related Disease

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Evaluating cell reprogramming, differentiation and conversion technologies in neuroscience

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(2016) Francisco Zurita et al.   Stem Cell Research

Effects of Feeder Cells on Dopaminergic Differentiation of Human Embryonic Stem Cells

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Development of a rapid screen for the endodermal differentiation potential of human pluripotent stem cell lines

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Oxidative stress in inherited mitochondrial diseases

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Valproic acid-induced hepatotoxicity in alpers syndrome is associated with mitochondrial permeability transition pore opening-dependent apoptotic sensitivity in an induced pluripotent stem cell model

(2015) Shengbiao Li et al.   HEPATOLOGY

The Role of Oxidative Stress and Antioxidants in Liver Diseases

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Neuroprotective effects of N-acetylcysteine amide on experimental focal penetrating brain injury in rats

(2015) Mattias Günther et al.   JOURNAL OF CLINICAL NEUROSCIENCE

Human iPSC-derived motoneurons harbouring TARDBP or C9ORF72 ALS mutations are dysfunctional despite maintaining viability

(2015) Anna-Claire Devlin et al.   Nature Communications

Molecular pathogenesis of polymerase gamma-related neurodegeneration

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N-acetylcysteine amide preserves mitochondrial bioenergetics and improves functional recovery following spinal trauma

(2014) Samir P. Patel et al.   EXPERIMENTAL NEUROLOGY

N-acetylcysteine amide confers neuroprotection, improves bioenergetics and behavioral outcome following TBI

(2014) Jignesh D. Pandya et al.   EXPERIMENTAL NEUROLOGY

Pluripotent stem cells in regenerative medicine: challenges and recent progress

(2014) Viviane Tabar et al.   NATURE REVIEWS GENETICS

In Vitro-Reconstituted Nucleoids Can Block Mitochondrial DNA Replication and Transcription

(2014) Géraldine Farge et al.   Cell Reports

Mesencephalic complex I deficiency does not correlate with parkinsonism in mitochondrial DNA maintenance disorders

(2013) Eino J. H. Palin et al.   BRAIN

Severe nigrostriatal degeneration without clinical parkinsonism in patients with polymerase gamma mutations

(2013) Charalampos Tzoulis et al.   BRAIN

N-Acetylcysteine amide: a derivative to fulfill the promises of N-Acetylcysteine

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Mitochondrial Biogenesis through Activation of Nuclear Signaling Proteins

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

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Efficient derivation of NPCs, spinal motor neurons and midbrain dopaminergic neurons from hESCs at 3% oxygen

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The Interplay Between Mitochondrial Dynamics and Mitophagy

(2010) Gilad Twig et al.   ANTIOXIDANTS & REDOX SIGNALING

A Novel POLG Gene Mutation in 4 Children With Alpers-like Hepatocerebral Syndromes

(2010) Bulent Kurt et al.   ARCHIVES OF NEUROLOGY

Derivation of 30 human embryonic stem cell lines—improving the quality

(2010) Susanne Ström et al.   IN VITRO CELLULAR & DEVELOPMENTAL BIOLOGY-ANIMAL

The antioxidant Trolox restores mitochondrial membrane potential and Ca2+-stimulated ATP production in human complex I deficiency

(2009) Felix Distelmaier et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

Mitochondria in the aetiology and pathogenesis of Parkinson's disease

(2008) Anthony HV Schapira   LANCET NEUROLOGY

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