Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.
Identifieur interne : 000134 ( Main/Exploration ); précédent : 000133; suivant : 000135Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.
Auteurs : Mariana Igoillo-Esteve ; Ana F. Oliveira ; Cristina Cosentino ; Federica Fantuzzi [Italie] ; Céline Demarez ; Sanna Toivonen ; Amélie Hu [Belgique] ; Satyan Chintawar [Belgique] ; Miguel Lopes ; Nathalie Pachera ; Ying Cai ; Baroj Abdulkarim ; Myriam Rai [Belgique] ; Lorella Marselli [Italie] ; Piero Marchetti [Italie] ; Mohammad Tariq [Italie] ; Jean-Christophe Jonas [Belgique] ; Marina Boscolo [Belgique] ; Massimo Pandolfo [Belgique] ; Décio L. Eizirik [États-Unis] ; Miriam Cnop [Belgique]Source :
- JCI insight [ 2379-3708 ] ; 2020.
Descripteurs français
- KwdFr :
- Adolescent (MeSH), Adulte (MeSH), Adulte d'âge moyen (MeSH), Animaux (MeSH), Ataxie de Friedreich (génétique), Ataxie de Friedreich (métabolisme), Ataxie de Friedreich (traitement médicamenteux), Cellules à insuline (métabolisme), Cervelet (anatomopathologie), Encéphale (anatomopathologie), Espèces réactives de l'oxygène (métabolisme), Expansion de trinucléotide répété (MeSH), Exénatide (pharmacologie), Exénatide (usage thérapeutique), Femelle (MeSH), Fer (métabolisme), Ganglions sensitifs des nerfs spinaux (anatomopathologie), Glucagon-like peptide 1 (analogues et dérivés), Glucagon-like peptide 1 (métabolisme), Humains (MeSH), Insuline (métabolisme), Jeune adulte (MeSH), Mitochondries (métabolisme), Modèles animaux de maladie humaine (MeSH), Mâle (MeSH), Protéines de liaison au fer (génétique), Protéines de liaison au fer (métabolisme), Régulation de l'expression des gènes (effets des médicaments et des substances chimiques), Souris (MeSH), Souris knockout (MeSH), Stress oxydatif (MeSH), Sujet âgé (MeSH), Techniques de knock-in de gènes (MeSH).
- MESH :
- analogues et dérivés : Glucagon-like peptide 1.
- anatomopathologie : Cervelet, Encéphale, Ganglions sensitifs des nerfs spinaux.
- effets des médicaments et des substances chimiques : Régulation de l'expression des gènes.
- génétique : Ataxie de Friedreich, Protéines de liaison au fer.
- métabolisme : Ataxie de Friedreich, Cellules à insuline, Espèces réactives de l'oxygène, Fer, Glucagon-like peptide 1, Insuline, Mitochondries, Protéines de liaison au fer.
- pharmacologie : Exénatide.
- traitement médicamenteux : Ataxie de Friedreich.
- usage thérapeutique : Exénatide.
- Adolescent, Adulte, Adulte d'âge moyen, Animaux, Expansion de trinucléotide répété, Femelle, Humains, Jeune adulte, Modèles animaux de maladie humaine, Mâle, Souris, Souris knockout, Stress oxydatif, Sujet âgé, Techniques de knock-in de gènes.
English descriptors
- KwdEn :
- Adolescent (MeSH), Adult (MeSH), Aged (MeSH), Animals (MeSH), Brain (pathology), Cerebellum (pathology), Disease Models, Animal (MeSH), Exenatide (pharmacology), Exenatide (therapeutic use), Female (MeSH), Friedreich Ataxia (drug therapy), Friedreich Ataxia (genetics), Friedreich Ataxia (metabolism), Ganglia, Spinal (pathology), Gene Expression Regulation (drug effects), Gene Knock-In Techniques (MeSH), Glucagon-Like Peptide 1 (analogs & derivatives), Glucagon-Like Peptide 1 (metabolism), Humans (MeSH), Insulin (metabolism), Insulin-Secreting Cells (metabolism), Iron (metabolism), Iron-Binding Proteins (genetics), Iron-Binding Proteins (metabolism), Male (MeSH), Mice (MeSH), Mice, Knockout (MeSH), Middle Aged (MeSH), Mitochondria (metabolism), Oxidative Stress (MeSH), Reactive Oxygen Species (metabolism), Trinucleotide Repeat Expansion (MeSH), Young Adult (MeSH).
- MESH :
- chemical , analogs & derivatives : Glucagon-Like Peptide 1.
- chemical , genetics : Iron-Binding Proteins.
- chemical , metabolism : Glucagon-Like Peptide 1, Insulin, Iron, Iron-Binding Proteins, Reactive Oxygen Species.
- chemical , pharmacology : Exenatide.
- drug effects : Gene Expression Regulation.
- drug therapy : Friedreich Ataxia.
- genetics : Friedreich Ataxia.
- metabolism : Friedreich Ataxia, Insulin-Secreting Cells, Mitochondria.
- pathology : Brain, Cerebellum, Ganglia, Spinal.
- chemical , therapeutic use : Exenatide.
- Adolescent, Adult, Aged, Animals, Disease Models, Animal, Female, Gene Knock-In Techniques, Humans, Male, Mice, Mice, Knockout, Middle Aged, Oxidative Stress, Trinucleotide Repeat Expansion, Young Adult.
Abstract
Friedreich ataxia is an autosomal recessive neurodegenerative disease associated with a high diabetes prevalence. No treatment is available to prevent or delay disease progression. Friedreich ataxia is caused by intronic GAA trinucleotide repeat expansions in the frataxin-encoding FXN gene that reduce frataxin expression, impair iron-sulfur cluster biogenesis, cause oxidative stress, and result in mitochondrial dysfunction and apoptosis. Here we examined the metabolic, neuroprotective, and frataxin-inducing effects of glucagon-like peptide-1 (GLP-1) analogs in in vivo and in vitro models and in patients with Friedreich ataxia. The GLP-1 analog exenatide improved glucose homeostasis of frataxin-deficient mice through enhanced insulin content and secretion in pancreatic β cells. Exenatide induced frataxin and iron-sulfur cluster-containing proteins in β cells and brain and was protective to sensory neurons in dorsal root ganglia. GLP-1 analogs also induced frataxin expression, reduced oxidative stress, and improved mitochondrial function in Friedreich ataxia patients' induced pluripotent stem cell-derived β cells and sensory neurons. The frataxin-inducing effect of exenatide was confirmed in a pilot trial in Friedreich ataxia patients, showing modest frataxin induction in platelets over a 5-week treatment course. Taken together, GLP-1 analogs improve mitochondrial function in frataxin-deficient cells and induce frataxin expression. Our findings identify incretin receptors as a therapeutic target in Friedreich ataxia.
DOI: 10.1172/jci.insight.134221
PubMed: 31877117
PubMed Central: PMC7098728
Affiliations:
- Belgique, Italie, États-Unis
- Indiana, Région de Bruxelles-Capitale, Toscane
- Bruxelles, Pise
- Université catholique de Louvain, Université de Pise, Université libre de Bruxelles
Links toward previous steps (curation, corpus...)
Le document en format XML
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<author><name sortKey="Chintawar, Satyan" sort="Chintawar, Satyan" uniqKey="Chintawar S" first="Satyan" last="Chintawar">Satyan Chintawar</name>
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<author><name sortKey="Lopes, Miguel" sort="Lopes, Miguel" uniqKey="Lopes M" first="Miguel" last="Lopes">Miguel Lopes</name>
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<author><name sortKey="Pachera, Nathalie" sort="Pachera, Nathalie" uniqKey="Pachera N" first="Nathalie" last="Pachera">Nathalie Pachera</name>
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<author><name sortKey="Cai, Ying" sort="Cai, Ying" uniqKey="Cai Y" first="Ying" last="Cai">Ying Cai</name>
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<author><name sortKey="Abdulkarim, Baroj" sort="Abdulkarim, Baroj" uniqKey="Abdulkarim B" first="Baroj" last="Abdulkarim">Baroj Abdulkarim</name>
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<author><name sortKey="Rai, Myriam" sort="Rai, Myriam" uniqKey="Rai M" first="Myriam" last="Rai">Myriam Rai</name>
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<author><name sortKey="Marselli, Lorella" sort="Marselli, Lorella" uniqKey="Marselli L" first="Lorella" last="Marselli">Lorella Marselli</name>
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<author><name sortKey="Marchetti, Piero" sort="Marchetti, Piero" uniqKey="Marchetti P" first="Piero" last="Marchetti">Piero Marchetti</name>
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<author><name sortKey="Tariq, Mohammad" sort="Tariq, Mohammad" uniqKey="Tariq M" first="Mohammad" last="Tariq">Mohammad Tariq</name>
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<author><name sortKey="Jonas, Jean Christophe" sort="Jonas, Jean Christophe" uniqKey="Jonas J" first="Jean-Christophe" last="Jonas">Jean-Christophe Jonas</name>
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<author><name sortKey="Boscolo, Marina" sort="Boscolo, Marina" uniqKey="Boscolo M" first="Marina" last="Boscolo">Marina Boscolo</name>
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<author><name sortKey="Pandolfo, Massimo" sort="Pandolfo, Massimo" uniqKey="Pandolfo M" first="Massimo" last="Pandolfo">Massimo Pandolfo</name>
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<country xml:lang="fr">Belgique</country>
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</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
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<author><name sortKey="Eizirik, Decio L" sort="Eizirik, Decio L" uniqKey="Eizirik D" first="Décio L" last="Eizirik">Décio L. Eizirik</name>
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<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
<affiliation wicri:level="2"><nlm:affiliation>Indiana Biosciences Research Institute, Indianapolis, Indiana, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Indiana Biosciences Research Institute, Indianapolis, Indiana</wicri:regionArea>
<placeName><region type="state">Indiana</region>
</placeName>
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</author>
<author><name sortKey="Cnop, Miriam" sort="Cnop, Miriam" uniqKey="Cnop M" first="Miriam" last="Cnop">Miriam Cnop</name>
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<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
<affiliation wicri:level="4"><nlm:affiliation>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
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<date when="2020">2020</date>
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<idno type="doi">10.1172/jci.insight.134221</idno>
<idno type="pmc">PMC7098728</idno>
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<sourceDesc><biblStruct><analytic><title xml:lang="en">Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.</title>
<author><name sortKey="Igoillo Esteve, Mariana" sort="Igoillo Esteve, Mariana" uniqKey="Igoillo Esteve M" first="Mariana" last="Igoillo-Esteve">Mariana Igoillo-Esteve</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Oliveira, Ana F" sort="Oliveira, Ana F" uniqKey="Oliveira A" first="Ana F" last="Oliveira">Ana F. Oliveira</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Cosentino, Cristina" sort="Cosentino, Cristina" uniqKey="Cosentino C" first="Cristina" last="Cosentino">Cristina Cosentino</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Fantuzzi, Federica" sort="Fantuzzi, Federica" uniqKey="Fantuzzi F" first="Federica" last="Fantuzzi">Federica Fantuzzi</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
<affiliation wicri:level="1"><nlm:affiliation>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma</wicri:regionArea>
<wicri:noRegion>Parma</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Demarez, Celine" sort="Demarez, Celine" uniqKey="Demarez C" first="Céline" last="Demarez">Céline Demarez</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Toivonen, Sanna" sort="Toivonen, Sanna" uniqKey="Toivonen S" first="Sanna" last="Toivonen">Sanna Toivonen</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Hu, Amelie" sort="Hu, Amelie" uniqKey="Hu A" first="Amélie" last="Hu">Amélie Hu</name>
<affiliation wicri:level="4"><nlm:affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
<author><name sortKey="Chintawar, Satyan" sort="Chintawar, Satyan" uniqKey="Chintawar S" first="Satyan" last="Chintawar">Satyan Chintawar</name>
<affiliation wicri:level="4"><nlm:affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
<author><name sortKey="Lopes, Miguel" sort="Lopes, Miguel" uniqKey="Lopes M" first="Miguel" last="Lopes">Miguel Lopes</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Pachera, Nathalie" sort="Pachera, Nathalie" uniqKey="Pachera N" first="Nathalie" last="Pachera">Nathalie Pachera</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Cai, Ying" sort="Cai, Ying" uniqKey="Cai Y" first="Ying" last="Cai">Ying Cai</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Abdulkarim, Baroj" sort="Abdulkarim, Baroj" uniqKey="Abdulkarim B" first="Baroj" last="Abdulkarim">Baroj Abdulkarim</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
</author>
<author><name sortKey="Rai, Myriam" sort="Rai, Myriam" uniqKey="Rai M" first="Myriam" last="Rai">Myriam Rai</name>
<affiliation wicri:level="4"><nlm:affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
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<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
<author><name sortKey="Marselli, Lorella" sort="Marselli, Lorella" uniqKey="Marselli L" first="Lorella" last="Marselli">Lorella Marselli</name>
<affiliation wicri:level="4"><nlm:affiliation>Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Clinical and Experimental Medicine, University of Pisa, Pisa</wicri:regionArea>
<placeName><settlement type="city">Pise</settlement>
<region nuts="2">Toscane</region>
</placeName>
<orgName type="university">Université de Pise</orgName>
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<author><name sortKey="Marchetti, Piero" sort="Marchetti, Piero" uniqKey="Marchetti P" first="Piero" last="Marchetti">Piero Marchetti</name>
<affiliation wicri:level="1"><nlm:affiliation>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma</wicri:regionArea>
<wicri:noRegion>Parma</wicri:noRegion>
</affiliation>
</author>
<author><name sortKey="Tariq, Mohammad" sort="Tariq, Mohammad" uniqKey="Tariq M" first="Mohammad" last="Tariq">Mohammad Tariq</name>
<affiliation wicri:level="4"><nlm:affiliation>Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.</nlm:affiliation>
<country xml:lang="fr">Italie</country>
<wicri:regionArea>Department of Clinical and Experimental Medicine, University of Pisa, Pisa</wicri:regionArea>
<placeName><settlement type="city">Pise</settlement>
<region nuts="2">Toscane</region>
</placeName>
<orgName type="university">Université de Pise</orgName>
</affiliation>
</author>
<author><name sortKey="Jonas, Jean Christophe" sort="Jonas, Jean Christophe" uniqKey="Jonas J" first="Jean-Christophe" last="Jonas">Jean-Christophe Jonas</name>
<affiliation wicri:level="4"><nlm:affiliation>Pole of Endocrinology, Diabetes and Nutrition, Institute of Experimental and Clinical Research, Université Catholique de Louvain, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Pole of Endocrinology, Diabetes and Nutrition, Institute of Experimental and Clinical Research, Université Catholique de Louvain, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université catholique de Louvain</orgName>
</affiliation>
</author>
<author><name sortKey="Boscolo, Marina" sort="Boscolo, Marina" uniqKey="Boscolo M" first="Marina" last="Boscolo">Marina Boscolo</name>
<affiliation wicri:level="4"><nlm:affiliation>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
<author><name sortKey="Pandolfo, Massimo" sort="Pandolfo, Massimo" uniqKey="Pandolfo M" first="Massimo" last="Pandolfo">Massimo Pandolfo</name>
<affiliation wicri:level="4"><nlm:affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
<author><name sortKey="Eizirik, Decio L" sort="Eizirik, Decio L" uniqKey="Eizirik D" first="Décio L" last="Eizirik">Décio L. Eizirik</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
<affiliation wicri:level="2"><nlm:affiliation>Indiana Biosciences Research Institute, Indianapolis, Indiana, USA.</nlm:affiliation>
<country xml:lang="fr">États-Unis</country>
<wicri:regionArea>Indiana Biosciences Research Institute, Indianapolis, Indiana</wicri:regionArea>
<placeName><region type="state">Indiana</region>
</placeName>
</affiliation>
</author>
<author><name sortKey="Cnop, Miriam" sort="Cnop, Miriam" uniqKey="Cnop M" first="Miriam" last="Cnop">Miriam Cnop</name>
<affiliation><nlm:affiliation>ULB Center for Diabetes Research and.</nlm:affiliation>
<wicri:noCountry code="no comma">ULB Center for Diabetes Research and.</wicri:noCountry>
</affiliation>
<affiliation wicri:level="4"><nlm:affiliation>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels, Belgium.</nlm:affiliation>
<country xml:lang="fr">Belgique</country>
<wicri:regionArea>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels</wicri:regionArea>
<placeName><settlement type="city">Bruxelles</settlement>
<region nuts="2">Région de Bruxelles-Capitale</region>
</placeName>
<orgName type="university">Université libre de Bruxelles</orgName>
</affiliation>
</author>
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<series><title level="j">JCI insight</title>
<idno type="eISSN">2379-3708</idno>
<imprint><date when="2020" type="published">2020</date>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent (MeSH)</term>
<term>Adult (MeSH)</term>
<term>Aged (MeSH)</term>
<term>Animals (MeSH)</term>
<term>Brain (pathology)</term>
<term>Cerebellum (pathology)</term>
<term>Disease Models, Animal (MeSH)</term>
<term>Exenatide (pharmacology)</term>
<term>Exenatide (therapeutic use)</term>
<term>Female (MeSH)</term>
<term>Friedreich Ataxia (drug therapy)</term>
<term>Friedreich Ataxia (genetics)</term>
<term>Friedreich Ataxia (metabolism)</term>
<term>Ganglia, Spinal (pathology)</term>
<term>Gene Expression Regulation (drug effects)</term>
<term>Gene Knock-In Techniques (MeSH)</term>
<term>Glucagon-Like Peptide 1 (analogs & derivatives)</term>
<term>Glucagon-Like Peptide 1 (metabolism)</term>
<term>Humans (MeSH)</term>
<term>Insulin (metabolism)</term>
<term>Insulin-Secreting Cells (metabolism)</term>
<term>Iron (metabolism)</term>
<term>Iron-Binding Proteins (genetics)</term>
<term>Iron-Binding Proteins (metabolism)</term>
<term>Male (MeSH)</term>
<term>Mice (MeSH)</term>
<term>Mice, Knockout (MeSH)</term>
<term>Middle Aged (MeSH)</term>
<term>Mitochondria (metabolism)</term>
<term>Oxidative Stress (MeSH)</term>
<term>Reactive Oxygen Species (metabolism)</term>
<term>Trinucleotide Repeat Expansion (MeSH)</term>
<term>Young Adult (MeSH)</term>
</keywords>
<keywords scheme="KwdFr" xml:lang="fr"><term>Adolescent (MeSH)</term>
<term>Adulte (MeSH)</term>
<term>Adulte d'âge moyen (MeSH)</term>
<term>Animaux (MeSH)</term>
<term>Ataxie de Friedreich (génétique)</term>
<term>Ataxie de Friedreich (métabolisme)</term>
<term>Ataxie de Friedreich (traitement médicamenteux)</term>
<term>Cellules à insuline (métabolisme)</term>
<term>Cervelet (anatomopathologie)</term>
<term>Encéphale (anatomopathologie)</term>
<term>Espèces réactives de l'oxygène (métabolisme)</term>
<term>Expansion de trinucléotide répété (MeSH)</term>
<term>Exénatide (pharmacologie)</term>
<term>Exénatide (usage thérapeutique)</term>
<term>Femelle (MeSH)</term>
<term>Fer (métabolisme)</term>
<term>Ganglions sensitifs des nerfs spinaux (anatomopathologie)</term>
<term>Glucagon-like peptide 1 (analogues et dérivés)</term>
<term>Glucagon-like peptide 1 (métabolisme)</term>
<term>Humains (MeSH)</term>
<term>Insuline (métabolisme)</term>
<term>Jeune adulte (MeSH)</term>
<term>Mitochondries (métabolisme)</term>
<term>Modèles animaux de maladie humaine (MeSH)</term>
<term>Mâle (MeSH)</term>
<term>Protéines de liaison au fer (génétique)</term>
<term>Protéines de liaison au fer (métabolisme)</term>
<term>Régulation de l'expression des gènes (effets des médicaments et des substances chimiques)</term>
<term>Souris (MeSH)</term>
<term>Souris knockout (MeSH)</term>
<term>Stress oxydatif (MeSH)</term>
<term>Sujet âgé (MeSH)</term>
<term>Techniques de knock-in de gènes (MeSH)</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="analogs & derivatives" xml:lang="en"><term>Glucagon-Like Peptide 1</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Iron-Binding Proteins</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="metabolism" xml:lang="en"><term>Glucagon-Like Peptide 1</term>
<term>Insulin</term>
<term>Iron</term>
<term>Iron-Binding Proteins</term>
<term>Reactive Oxygen Species</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="pharmacology" xml:lang="en"><term>Exenatide</term>
</keywords>
<keywords scheme="MESH" qualifier="analogues et dérivés" xml:lang="fr"><term>Glucagon-like peptide 1</term>
</keywords>
<keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Cervelet</term>
<term>Encéphale</term>
<term>Ganglions sensitifs des nerfs spinaux</term>
</keywords>
<keywords scheme="MESH" qualifier="drug effects" xml:lang="en"><term>Gene Expression Regulation</term>
</keywords>
<keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Friedreich Ataxia</term>
</keywords>
<keywords scheme="MESH" qualifier="effets des médicaments et des substances chimiques" xml:lang="fr"><term>Régulation de l'expression des gènes</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Friedreich Ataxia</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Ataxie de Friedreich</term>
<term>Protéines de liaison au fer</term>
</keywords>
<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Friedreich Ataxia</term>
<term>Insulin-Secreting Cells</term>
<term>Mitochondria</term>
</keywords>
<keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Ataxie de Friedreich</term>
<term>Cellules à insuline</term>
<term>Espèces réactives de l'oxygène</term>
<term>Fer</term>
<term>Glucagon-like peptide 1</term>
<term>Insuline</term>
<term>Mitochondries</term>
<term>Protéines de liaison au fer</term>
</keywords>
<keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Brain</term>
<term>Cerebellum</term>
<term>Ganglia, Spinal</term>
</keywords>
<keywords scheme="MESH" qualifier="pharmacologie" xml:lang="fr"><term>Exénatide</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Exenatide</term>
</keywords>
<keywords scheme="MESH" qualifier="traitement médicamenteux" xml:lang="fr"><term>Ataxie de Friedreich</term>
</keywords>
<keywords scheme="MESH" qualifier="usage thérapeutique" xml:lang="fr"><term>Exénatide</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Adult</term>
<term>Aged</term>
<term>Animals</term>
<term>Disease Models, Animal</term>
<term>Female</term>
<term>Gene Knock-In Techniques</term>
<term>Humans</term>
<term>Male</term>
<term>Mice</term>
<term>Mice, Knockout</term>
<term>Middle Aged</term>
<term>Oxidative Stress</term>
<term>Trinucleotide Repeat Expansion</term>
<term>Young Adult</term>
</keywords>
<keywords scheme="MESH" xml:lang="fr"><term>Adolescent</term>
<term>Adulte</term>
<term>Adulte d'âge moyen</term>
<term>Animaux</term>
<term>Expansion de trinucléotide répété</term>
<term>Femelle</term>
<term>Humains</term>
<term>Jeune adulte</term>
<term>Modèles animaux de maladie humaine</term>
<term>Mâle</term>
<term>Souris</term>
<term>Souris knockout</term>
<term>Stress oxydatif</term>
<term>Sujet âgé</term>
<term>Techniques de knock-in de gènes</term>
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<front><div type="abstract" xml:lang="en">Friedreich ataxia is an autosomal recessive neurodegenerative disease associated with a high diabetes prevalence. No treatment is available to prevent or delay disease progression. Friedreich ataxia is caused by intronic GAA trinucleotide repeat expansions in the frataxin-encoding FXN gene that reduce frataxin expression, impair iron-sulfur cluster biogenesis, cause oxidative stress, and result in mitochondrial dysfunction and apoptosis. Here we examined the metabolic, neuroprotective, and frataxin-inducing effects of glucagon-like peptide-1 (GLP-1) analogs in in vivo and in vitro models and in patients with Friedreich ataxia. The GLP-1 analog exenatide improved glucose homeostasis of frataxin-deficient mice through enhanced insulin content and secretion in pancreatic β cells. Exenatide induced frataxin and iron-sulfur cluster-containing proteins in β cells and brain and was protective to sensory neurons in dorsal root ganglia. GLP-1 analogs also induced frataxin expression, reduced oxidative stress, and improved mitochondrial function in Friedreich ataxia patients' induced pluripotent stem cell-derived β cells and sensory neurons. The frataxin-inducing effect of exenatide was confirmed in a pilot trial in Friedreich ataxia patients, showing modest frataxin induction in platelets over a 5-week treatment course. Taken together, GLP-1 analogs improve mitochondrial function in frataxin-deficient cells and induce frataxin expression. Our findings identify incretin receptors as a therapeutic target in Friedreich ataxia.</div>
</front>
</TEI>
<pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">31877117</PMID>
<DateCompleted><Year>2020</Year>
<Month>11</Month>
<Day>20</Day>
</DateCompleted>
<DateRevised><Year>2020</Year>
<Month>11</Month>
<Day>20</Day>
</DateRevised>
<Article PubModel="Electronic"><Journal><ISSN IssnType="Electronic">2379-3708</ISSN>
<JournalIssue CitedMedium="Internet"><Volume>5</Volume>
<Issue>2</Issue>
<PubDate><Year>2020</Year>
<Month>01</Month>
<Day>30</Day>
</PubDate>
</JournalIssue>
<Title>JCI insight</Title>
<ISOAbbreviation>JCI Insight</ISOAbbreviation>
</Journal>
<ArticleTitle>Exenatide induces frataxin expression and improves mitochondrial function in Friedreich ataxia.</ArticleTitle>
<ELocationID EIdType="doi" ValidYN="Y">10.1172/jci.insight.134221</ELocationID>
<ELocationID EIdType="pii" ValidYN="Y">134221</ELocationID>
<Abstract><AbstractText>Friedreich ataxia is an autosomal recessive neurodegenerative disease associated with a high diabetes prevalence. No treatment is available to prevent or delay disease progression. Friedreich ataxia is caused by intronic GAA trinucleotide repeat expansions in the frataxin-encoding FXN gene that reduce frataxin expression, impair iron-sulfur cluster biogenesis, cause oxidative stress, and result in mitochondrial dysfunction and apoptosis. Here we examined the metabolic, neuroprotective, and frataxin-inducing effects of glucagon-like peptide-1 (GLP-1) analogs in in vivo and in vitro models and in patients with Friedreich ataxia. The GLP-1 analog exenatide improved glucose homeostasis of frataxin-deficient mice through enhanced insulin content and secretion in pancreatic β cells. Exenatide induced frataxin and iron-sulfur cluster-containing proteins in β cells and brain and was protective to sensory neurons in dorsal root ganglia. GLP-1 analogs also induced frataxin expression, reduced oxidative stress, and improved mitochondrial function in Friedreich ataxia patients' induced pluripotent stem cell-derived β cells and sensory neurons. The frataxin-inducing effect of exenatide was confirmed in a pilot trial in Friedreich ataxia patients, showing modest frataxin induction in platelets over a 5-week treatment course. Taken together, GLP-1 analogs improve mitochondrial function in frataxin-deficient cells and induce frataxin expression. Our findings identify incretin receptors as a therapeutic target in Friedreich ataxia.</AbstractText>
</Abstract>
<AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Igoillo-Esteve</LastName>
<ForeName>Mariana</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Oliveira</LastName>
<ForeName>Ana F</ForeName>
<Initials>AF</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Cosentino</LastName>
<ForeName>Cristina</ForeName>
<Initials>C</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Fantuzzi</LastName>
<ForeName>Federica</ForeName>
<Initials>F</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma, Italy.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Demarez</LastName>
<ForeName>Céline</ForeName>
<Initials>C</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Toivonen</LastName>
<ForeName>Sanna</ForeName>
<Initials>S</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Hu</LastName>
<ForeName>Amélie</ForeName>
<Initials>A</Initials>
<AffiliationInfo><Affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Chintawar</LastName>
<ForeName>Satyan</ForeName>
<Initials>S</Initials>
<AffiliationInfo><Affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Lopes</LastName>
<ForeName>Miguel</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Pachera</LastName>
<ForeName>Nathalie</ForeName>
<Initials>N</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Cai</LastName>
<ForeName>Ying</ForeName>
<Initials>Y</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Abdulkarim</LastName>
<ForeName>Baroj</ForeName>
<Initials>B</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Rai</LastName>
<ForeName>Myriam</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Marselli</LastName>
<ForeName>Lorella</ForeName>
<Initials>L</Initials>
<AffiliationInfo><Affiliation>Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Marchetti</LastName>
<ForeName>Piero</ForeName>
<Initials>P</Initials>
<AffiliationInfo><Affiliation>Endocrinology and Metabolism, Department of Medicine and Surgery, University of Parma, Parma, Italy.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Tariq</LastName>
<ForeName>Mohammad</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Jonas</LastName>
<ForeName>Jean-Christophe</ForeName>
<Initials>JC</Initials>
<AffiliationInfo><Affiliation>Pole of Endocrinology, Diabetes and Nutrition, Institute of Experimental and Clinical Research, Université Catholique de Louvain, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Boscolo</LastName>
<ForeName>Marina</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Pandolfo</LastName>
<ForeName>Massimo</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>Laboratory of Experimental Neurology, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Eizirik</LastName>
<ForeName>Décio L</ForeName>
<Initials>DL</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Indiana Biosciences Research Institute, Indianapolis, Indiana, USA.</Affiliation>
</AffiliationInfo>
</Author>
<Author ValidYN="Y"><LastName>Cnop</LastName>
<ForeName>Miriam</ForeName>
<Initials>M</Initials>
<AffiliationInfo><Affiliation>ULB Center for Diabetes Research and.</Affiliation>
</AffiliationInfo>
<AffiliationInfo><Affiliation>Division of Endocrinology, Erasmus Hospital, Université Libre de Bruxelles, Brussels, Belgium.</Affiliation>
</AffiliationInfo>
</Author>
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<Language>eng</Language>
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</PublicationTypeList>
<ArticleDate DateType="Electronic"><Year>2020</Year>
<Month>01</Month>
<Day>30</Day>
</ArticleDate>
</Article>
<MedlineJournalInfo><Country>United States</Country>
<MedlineTA>JCI Insight</MedlineTA>
<NlmUniqueID>101676073</NlmUniqueID>
<ISSNLinking>2379-3708</ISSNLinking>
</MedlineJournalInfo>
<ChemicalList><Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D007328">Insulin</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D033862">Iron-Binding Proteins</NameOfSubstance>
</Chemical>
<Chemical><RegistryNumber>0</RegistryNumber>
<NameOfSubstance UI="D017382">Reactive Oxygen Species</NameOfSubstance>
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<NameOfSubstance UI="C098527">frataxin</NameOfSubstance>
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<Chemical><RegistryNumber>9P1872D4OL</RegistryNumber>
<NameOfSubstance UI="D000077270">Exenatide</NameOfSubstance>
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<Chemical><RegistryNumber>E1UOL152H7</RegistryNumber>
<NameOfSubstance UI="D007501">Iron</NameOfSubstance>
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<CitationSubset>IM</CitationSubset>
<MeshHeadingList><MeshHeading><DescriptorName UI="D000293" MajorTopicYN="N">Adolescent</DescriptorName>
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<MeshHeading><DescriptorName UI="D000328" MajorTopicYN="N">Adult</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D000368" MajorTopicYN="N">Aged</DescriptorName>
</MeshHeading>
<MeshHeading><DescriptorName UI="D000818" MajorTopicYN="N">Animals</DescriptorName>
</MeshHeading>
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<MeshHeading><DescriptorName UI="D002531" MajorTopicYN="N">Cerebellum</DescriptorName>
<QualifierName UI="Q000473" MajorTopicYN="N">pathology</QualifierName>
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