Unveiling a common mechanism of apoptosis in β-cells and neurons in Friedreich's ataxia

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JunB protects β-cells from lipotoxicity via the XBP1–AKT pathway

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Diabetes in Friedreich Ataxia

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Friedreich Ataxia: Neuropathology Revised

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NADPH Oxidase NOX2 Defines a New Antagonistic Role for Reactive Oxygen Species and cAMP/PKA in the Regulation of Insulin Secretion

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Mitochondrial oxidative stress contributes differently to rat pancreatic islet cell apoptosis and insulin secretory defects after prolonged culture in a low non-stimulating glucose concentration

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Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich's ataxia

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Friedreich Ataxia

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Exendin-4 Ameliorates Motor Neuron Degeneration in Cellular and Animal Models of Amyotrophic Lateral Sclerosis

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Dynamic measurements of mitochondrial hydrogen peroxide concentration and glutathione redox state in rat pancreatic β-cells using ratiometric fluorescent proteins: confounding effects of pH with HyPer but not roGFP1

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Repeated administration of exendin-4 reduces focal cerebral ischemia-induced infarction in rats

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Neuroprotective properties of GLP-1: theoretical and practical applications

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Silencing of frataxin gene expression triggers p53-dependent apoptosis in human neuron-like cells

(2011) G. M. Palomo et al.   HUMAN MOLECULAR GENETICS

Cytokines Tumor Necrosis Factor-α and Interferon-γ Induce Pancreatic β-Cell Apoptosis through STAT1-mediated Bim Protein Activation

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Cytokine toxicity in insulin-producing cells is mediated by nitro-oxidative stress-induced hydroxyl radical formation in mitochondria

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(2011) Arnulf H. Koeppen   JOURNAL OF THE NEUROLOGICAL SCIENCES

Bcl-2 proteins in diabetes: mitochondrial pathways of β-cell death and dysfunction

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Friedreich's Ataxia Induced Pluripotent Stem Cells Model Intergenerational GAA⋅TTC Triplet Repeat Instability

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Superior Cerebellar Peduncle Atrophy in Friedreich’s Ataxia Correlates with Disease Symptoms

(2010) Hamed Akhlaghi et al.   CEREBELLUM

Palmitate induces a pro-inflammatory response in human pancreatic islets that mimics CCL2 expression by beta cells in type 2 diabetes

(2010) M. Igoillo-Esteve et al.   DIABETOLOGIA

Does oxidative stress contribute to the pathology of Friedreich’s ataxia? A radical question

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MnTMPyP, a superoxide dismutase/catalase mimetic, decreases inflammatory indices in ischemic acute kidney injury

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p53 Up-regulated Modulator of Apoptosis (PUMA) Activation Contributes to Pancreatic β-Cell Apoptosis Induced by Proinflammatory Cytokines and Endoplasmic Reticulum Stress

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Enhanced Signaling Downstream of Ribonucleic Acid-Activated Protein Kinase-Like Endoplasmic Reticulum Kinase Potentiates Lipotoxic Endoplasmic Reticulum Stress in Human Islets

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Generation of Induced Pluripotent Stem Cell Lines from Friedreich Ataxia Patients

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Signaling by IL-1β+IFN-γ and ER stress converge on DP5/Hrk activation: a novel mechanism for pancreatic β-cell apoptosis

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PTPN2, a Candidate Gene for Type 1 Diabetes, Modulates Interferon- -Induced Pancreatic  -Cell Apoptosis

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Glucagon-Like Peptide-1 Agonists Protect Pancreatic β-Cells From Lipotoxic Endoplasmic Reticulum Stress Through Upregulation of BiP and JunB

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Control of mitochondrial apoptosis by the Bcl-2 family

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Friedreich ataxia: The clinical picture

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Stepwise Activation of BAX and BAK by tBID, BIM, and PUMA Initiates Mitochondrial Apoptosis

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Cell Death

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GLP-1 receptor stimulation preserves primary cortical and dopaminergic neurons in cellular and rodent models of stroke and Parkinsonism

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Exendin-4 Improves Glycemic Control, Ameliorates Brain and Pancreatic Pathologies, and Extends Survival in a Mouse Model of Huntington's Disease

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Hydrogen peroxide scavenging rescues frataxin deficiency in a Drosophila model of Friedreich's ataxia

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Drug Insight: antioxidant therapy in inherited ataxias

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