Journal article
Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons
International journal of molecular sciences, v 14(4), pp 7853-7865
10 Apr 2013
PMID: 23574943
Featured in Collection : UN Sustainable Development Goals @ Drexel
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 findings may help to focus on this promising target, especially in its emerging role in the neuroprotective response.
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Details
- Title
- Frataxin Deficiency Leads to Reduced Expression and Impaired Translocation of NF-E2-Related Factor (Nrf2) in Cultured Motor Neurons
- Creators
- Valentina D'Oria - IRCCS, Bambino Gesu Childrens Hosp, Res Labs, Confocal Microscopy Core Facil, I-00165 Rome, ItalyStefania Petrini - Bambino Gesù Children's HospitalLorena Travaglini - Bambino Gesù Children's HospitalChiara Priori - Bambino Gesù Children's HospitalEmanuela Piermarini - Bambino Gesù Children's HospitalSara Petrillo - Bambino Gesù Children's HospitalBarbara Carletti - IRCCS, Bambino Gesu Childrens Hosp, Unit Neuromuscular & Neurodegenerat Dis, I-00165 Rome, ItalyEnrico Bertini - Bambino Gesù Children's HospitalFiorella Piemonte - Bambino Gesù Children's Hospital
- Publication Details
- International journal of molecular sciences, v 14(4), pp 7853-7865
- Publisher
- Mdpi
- Number of pages
- 13
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Neurobiology and Anatomy
- Web of Science ID
- WOS:000318017100069
- Scopus ID
- 2-s2.0-84876310656
- Other Identifier
- 991021900032304721
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InCites Highlights
Data related to this publication, from InCites Benchmarking & Analytics tool:
- Web of Science research areas
- Biochemistry & Molecular Biology
- Chemistry, Multidisciplinary