{"content"=>"Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in aModel of Friedreich's Ataxia.", "i"=>{"content"=>"Drosophila"}} (original) (raw)

Mitofusin-Dependent ER Stress Triggers Glial Dysfunction and Nervous System Degeneration in a Drosophila Model of Friedreich's Ataxia

Juan Antonio Navarro Langa

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Frataxin is Reduced in Friedreich Ataxia Patients and is Associated with Mitochondrial Membranes

Paul TRouillas

Human Molecular Genetics, 1997

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Extra-mitochondrial mouse frataxin and its implications for mouse models of Friedreich’s ataxia

Liwei Weng

Scientific Reports, 2020

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Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells

Jean-michel Camadro

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Reduction in frataxin causes progressive accumulation of mitochondrial damage

Grazia Isaya

Human Molecular Genetics, 2003

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Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant

Pilar Cabo

The FASEB Journal, 2005

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Frataxin interacts functionally with mitochondrial electron transport chain proteins

Pilar Cabo

Human Molecular Genetics, 2005

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Frataxin is essential for extramitochondrial Fe S cluster proteins in mammalian tissues

Marie Wattenhofer-Donzé

Human Molecular Genetics, 2007

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Frataxin expression rescues mitochondrial dysfunctions in FRDA cells

Cinzia Gellera

Human Molecular Genetics, 2001

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Causative role of oxidative stress in a Drosophila model of Friedreich ataxia

Maria Molto, Jose Llorens, Juan Antonio Navarro Langa

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Neurodegeneration in Friedreich's ataxia: from defective frataxin to oxidative stress

renata carolina albuquerque santos

Oxidative medicine and cellular longevity, 2013

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Friedreich's ataxia protein: phylogenetic evidence for mitochondrial dysfunction

Giovanna Musco

Trends in Neurosciences, 1996

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The pathogenesis of Friedreich ataxia and the structure and function of frataxin

Annalisa Pastore

Journal of Neurology, 2009

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Drosophila melanogaster Models of Friedreich’s Ataxia

Juan Antonio Navarro Langa

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Frataxin and mitochondrial FeS cluster biogenesis

Timothy Stemmler

Journal of Biological …, 2010

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Frataxin activates mitochondrial energy conversion and oxidative phosphorylation

Michael II

Proceedings of The National Academy of Sciences, 2000

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Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins

Bertrand Friguet

FEBS Journal, 2009

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Mitochondrial energy imbalance and lipid peroxidation cause cell death in Friedreich’s ataxia

iain hargreaves

Cell Death & Disease, 2016

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Mitochondrial iron detoxification is a primary function of frataxin that limits oxidative damage and preserves cell longevity

Gloria Ferreira

Human Molecular Genetics, 2005

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Mitochondrial dysfunction induced by frataxin deficiency is associated with cellular senescence and abnormal calcium metabolism

Pilar Cabo

Frontiers in Cellular Neuroscience, 2014

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Simultaneous Quantification of Mitochondrial Mature Frataxin and Extra-Mitochondrial Frataxin Isoform E in Friedreich’s Ataxia Blood

Liwei Weng

Frontiers in Neuroscience

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Frataxin deficiency alters heme pathway transcripts and decreases mitochondrial heme metabolites in mammalian cells

Eleonora Napoli

Human Molecular Genetics, 2005

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Maturation of wild-type and mutated frataxin by the mitochondrial processing peptidase

Victoria Campuzano

Human Molecular Genetics, 1998

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Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration

manish jaiswal

eLife, 2016

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Mitochondrial pathophysiology in Friedreich's ataxia

Pilar Cabo

Journal of Neurochemistry, 2013

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Preventing the ubiquitin-proteasome-dependent degradation of frataxin, the protein defective in Friedreich's ataxia

Natascia Ventura

Human Molecular Genetics, 2011

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Frataxin Silencing Inactivates Mitochondrial Complex I in NSC34 Motoneuronal Cells and Alters Glutathione Homeostasis

Marco Sparaco

International Journal of Molecular Sciences, 2014

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Overexpression of human and fly frataxins in Drosophila provokes deleterious effects at biochemical, physiological and developmental levels

Juan Antonio Navarro Langa

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Iron-dependent regulation of frataxin expression: implications for treatment of Friedreich ataxia

Dung Ha

Human Molecular Genetics, 2008

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The N-terminus of mature human frataxin is intrinsically unfolded

Salvatore Adinolfi

FEBS Journal, 2009

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