(Q38924043)

7 September 2017

title

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

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7 September 2017

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8

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7 September 2017

Baroj Abdulkarim

5

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Satyan Chintawar

Satyan Chintawar

6

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Jean-Christophe Jonas

Jean-Christophe Jonas

9

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Miriam Cnop

Miriam Cnop

12

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Lorella Marselli

Lorella Marselli

7

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Ewa Gurgul-Convey

Ewa Gurgul-Convey

2

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Mariana Igoillo-Esteve

Mariana Igoillo-Esteve

1

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author name string

Amélie Hu

3

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7 September 2017

Laila Romagueira Bichara Dos Santos

4

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7 September 2017

Décio L Eizirik

10

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7 September 2017

Massimo Pandolfo

11

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7 September 2017

publication date

30 December 2014

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7 September 2017

published in

Human Molecular Genetics

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7 September 2017

volume

24

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7 September 2017

issue

8

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7 September 2017

page(s)

2274-2286

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Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion

7 September 2017

cites work

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Clinical and genetic abnormalities in patients with Friedreich's ataxia

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Glucose metabolism alterations in Friedreich's ataxia

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Superior cerebellar peduncle atrophy in Friedreich's ataxia correlates with disease symptoms.

21 January 2018

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Friedreich ataxia: neuropathology revised

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Central role and mechanisms of β-cell dysfunction and death in friedreich ataxia-associated diabetes

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Diabetes in Friedreich ataxia

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Aconitase and mitochondrial iron-sulphur protein deficiency in Friedreich ataxia

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Frataxin acts as an iron chaperone protein to modulate mitochondrial aconitase activity

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

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

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

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Control of mitochondrial apoptosis by the Bcl-2 family

21 January 2018

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Friedreich's ataxia induced pluripotent stem cells model intergenerational GAA⋅TTC triplet repeat instability

21 January 2018

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Generation of induced pluripotent stem cell lines from Friedreich ataxia patients.

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Role of mismatch repair enzymes in GAA·TTC triplet-repeat expansion in Friedreich ataxia induced pluripotent stem cells

21 January 2018

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

21 January 2018

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The Friedreich's ataxia mutation confers cellular sensitivity to oxidant stress which is rescued by chelators of iron and calcium and inhibitors of apoptosis

21 January 2018

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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

21 January 2018

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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.

21 January 2018

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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

21 January 2018

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MnTMPyP, a cell-permeant SOD mimetic, reduces oxidative stress and apoptosis following renal ischemia-reperfusion

21 January 2018

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Stepwise activation of BAX and BAK by tBID, BIM, and PUMA initiates mitochondrial apoptosis.

21 January 2018

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The BCL-2 protein family: opposing activities that mediate cell death

21 January 2018

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BimS-induced apoptosis requires mitochondrial localization but not interaction with anti-apoptotic Bcl-2 proteins

21 January 2018

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The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues

21 January 2018

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Frataxin deficiency in pancreatic islets causes diabetes due to loss of beta cell mass

21 January 2018

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Friedreich ataxia: new pathways

21 January 2018

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iPSC-derived β cells model diabetes due to glucokinase deficiency

21 January 2018

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Why vitamin E therapy fails for treatment of Alzheimer's disease

21 January 2018

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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.

21 January 2018

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Neuroprotective properties of GLP-1: theoretical and practical applications

21 January 2018

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Exendin-4 ameliorates motor neuron degeneration in cellular and animal models of amyotrophic lateral sclerosis

21 January 2018

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The diabetes drug liraglutide prevents degenerative processes in a mouse model of Alzheimer's disease.

21 January 2018

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

21 January 2018

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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

21 January 2018

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https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Glucagon-like peptide-1 receptor activation reduces ischaemic brain damage following stroke in Type 2 diabetic rats

21 January 2018

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Establishment of 2-mercaptoethanol-dependent differentiated insulin-secreting cell lines

21 January 2018

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Prolonged exposure to free fatty acids has cytostatic and pro-apoptotic effects on human pancreatic islets: evidence that beta-cell death is caspase mediated, partially dependent on ceramide pathway, and Bcl-2 regulated.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

PTPN2, a candidate gene for type 1 diabetes, modulates interferon-gamma-induced pancreatic beta-cell apoptosis

21 January 2018

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Glucagon-like peptide-1 agonists protect pancreatic beta-cells from lipotoxic endoplasmic reticulum stress through upregulation of BiP and JunB.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Inverse relationship between cytotoxicity of free fatty acids in pancreatic islet cells and cellular triglyceride accumulation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Initiation and execution of lipotoxic ER stress in pancreatic beta-cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Mitochondrial catalase overexpression protects insulin-producing cells against toxicity of reactive oxygen species and proinflammatory cytokines.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

p53 up-regulated modulator of apoptosis (PUMA) activation contributes to pancreatic beta-cell apoptosis induced by proinflammatory cytokines and endoplasmic reticulum stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Cytokines tumor necrosis factor-α and interferon-γ induce pancreatic β-cell apoptosis through STAT1-mediated Bim protein activation

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Enhanced signaling downstream of ribonucleic Acid-activated protein kinase-like endoplasmic reticulum kinase potentiates lipotoxic endoplasmic reticulum stress in human islets.

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

Toll-like receptor 3 and STAT-1 contribute to double-stranded RNA+ interferon-gamma-induced apoptosis in primary pancreatic beta-cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

tRNA methyltransferase homolog gene TRMT10A mutation in young onset diabetes and primary microcephaly in humans

21 January 2018

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDU745

21 January 2018

Identifiers

DOI

10.1093/HMG/DDU745

1 reference

7 September 2017

1 reference