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Author Petrini, Stefania ♦ Travaglini, Lorena ♦ Priori, Chiara ♦ Piermarini, Emanuela ♦ Petrillo, Sara ♦ Carletti, Barbara ♦ Bertini, Enrico ♦ Piemonte, Fiorella
Source CiteSeerX
Content type Text
File Format PDF
Subject Domain (in DDC) Computer science, information & general works ♦ Data processing & computer science
Subject Keyword Cultured Motor Neuron ♦ Frataxin Deficiency Lead ♦ Nf-e2-related Factor ♦ Impaired Translocation ♦ Reduced Expression ♦ Neurodegenerative Disease ♦ Mitochondrial Protein ♦ Iron Homeostasis ♦ Cellular Susceptibility ♦ Defective Activation ♦ Antioxidant Cellular Response ♦ Nrf2-are Pathway ♦ Frataxin Gene ♦ Decreased Expression ♦ Confocal Microscopy ♦ Silenced Neuron ♦ Frda Neuron ♦ Transcription Factor Nf-e2-related Factor ♦ Antioxidant Response Element ♦ Frataxin-silenced Neuron
Abstract Abstract: Oxidative stress has been implicated in the pathogenesis of Friedreich’s Ataxia (FRDA), a neurodegenerative disease caused by the decreased expression of frataxin, a mitochondrial protein responsible of iron homeostasis. Under conditions of oxidative stress, the activation of the transcription factor NF-E2-related factor (Nrf2) triggers the antioxidant cellular response by inducing antioxidant response element (ARE) driven genes. Increasing evidence supports a role for the Nrf2-ARE pathway in neurodegenerative diseases. In this study, we analyzed the expression and the distribution of Nrf2 in silenced neurons for frataxin gene. Decreased Nrf2 mRNA content and a defective activation after treatment with pro-oxidants have been evidenced in frataxin-silenced neurons by RT-PCR and confocal microscopy. The loss of Nrf2 in FRDA may greatly enhance the cellular susceptibility to oxidative stress and make FRDA neurons more vulnerable to injury. Our
Educational Role Student ♦ Teacher
Age Range above 22 year
Educational Use Research
Education Level UG and PG ♦ Career/Technical Study
Learning Resource Type Article
Publisher Date 2013-01-01