Gene expression profiling in frataxin deficient mice: Microarray evidence for significant expression changes without detectable neurodegeneration
Friedreich's ataxia (FRDA) is caused by reduction of frataxin levels to 5–35%. To better understand the biochemical sequelae of frataxin reduction, in absence of the confounding effects of neurodegeneration, we studied the gene expression profile of a mouse model expressing 25–36% of the normal...
| Published in: | Neurobiology of Disease |
|---|---|
| Main Authors: | Giovanni Coppola, Sang-Hyun Choi, Manuela M. Santos, Carlos J. Miranda, Dmitri Tentler, Eric M. Wexler, Massimo Pandolfo, Daniel H. Geschwind |
| Format: | Article |
| Language: | English |
| Published: |
Elsevier
2006-05-01
|
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S096999610500313X |
Similar Items
Inducible and reversible phenotypes in a novel mouse model of Friedreich’s Ataxia
by: Vijayendran Chandran, et al.
Published: (2017-12-01)
by: Vijayendran Chandran, et al.
Published: (2017-12-01)
Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin
by: Daniel Fil, et al.
Published: (2023-02-01)
by: Daniel Fil, et al.
Published: (2023-02-01)
Glial cell activation precedes neurodegeneration in the cerebellar cortex of the YG8–800 murine model of Friedreich ataxia
by: Andrés Vicente-Acosta, et al.
Published: (2024-10-01)
by: Andrés Vicente-Acosta, et al.
Published: (2024-10-01)
Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency
by: Nicole M. Sayles, et al.
Published: (2023-10-01)
by: Nicole M. Sayles, et al.
Published: (2023-10-01)
Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration
by: Kuchuan Chen, et al.
Published: (2016-06-01)
by: Kuchuan Chen, et al.
Published: (2016-06-01)
Frataxin deficiency unveils cell-context dependent actions of insulin-like growth factor I on neurons
by: Franco Carolina, et al.
Published: (2012-10-01)
by: Franco Carolina, et al.
Published: (2012-10-01)
A peptide derived from TID1S rescues frataxin deficiency and mitochondrial defects in FRDA cellular models
by: Yi Na Dong, et al.
Published: (2024-03-01)
by: Yi Na Dong, et al.
Published: (2024-03-01)
Anti-gene oligonucleotides targeting Friedreich’s ataxia expanded GAA⋅TTC repeats increase Frataxin expression
by: Negin Mozafari, et al.
Published: (2025-06-01)
by: Negin Mozafari, et al.
Published: (2025-06-01)
Friedreichs ataxia in a diabetic patient
by: Inna Igorevna Klefortova, et al.
Published: (2010-06-01)
by: Inna Igorevna Klefortova, et al.
Published: (2010-06-01)
Protoporphyrin IX Binds to Iron(II)-Loaded and to Zinc-Loaded Human Frataxin
by: Ganeko Bernardo-Seisdedos, et al.
Published: (2023-01-01)
by: Ganeko Bernardo-Seisdedos, et al.
Published: (2023-01-01)
A Combined Spectroscopic and In Silico Approach to Evaluate the Interaction of Human Frataxin with Mitochondrial Superoxide Dismutase
by: Davide Doni, et al.
Published: (2021-11-01)
by: Davide Doni, et al.
Published: (2021-11-01)
A new FRDA mouse model [Fxnnull:YG8s(GAA) > 800] with more than 800 GAA repeats
by: Ester Kalef-Ezra, et al.
Published: (2023-01-01)
by: Ester Kalef-Ezra, et al.
Published: (2023-01-01)
Friedreich's Ataxia – A Clinical Diagnosis
by: Md. Fekarul Islam, et al.
Published: (2015-01-01)
by: Md. Fekarul Islam, et al.
Published: (2015-01-01)
Comparison of Two Different PCR-based Methods for Detection of GAA Expansions in Frataxin Gene
by: Mona ENTEZAM, et al.
Published: (2017-02-01)
by: Mona ENTEZAM, et al.
Published: (2017-02-01)
Difficulties translating antisense-mediated activation of Frataxin expression from cell culture to mice
by: Audrius Kilikevicius, et al.
Published: (2022-12-01)
by: Audrius Kilikevicius, et al.
Published: (2022-12-01)
An RNA-seq study in Friedreich ataxia patients identified hsa-miR-148a-3p as a putative prognostic biomarker of the disease
by: Chiara Vancheri, et al.
Published: (2024-05-01)
by: Chiara Vancheri, et al.
Published: (2024-05-01)
Alpha–lipoic acid supplementation improves pathological alterations in cellular models of Friedreich ataxia
by: Marta Talaverón-Rey, et al.
Published: (2025-08-01)
by: Marta Talaverón-Rey, et al.
Published: (2025-08-01)
Skeletal muscle proteome analysis underpins multifaceted mitochondrial dysfunction in Friedreich’s ataxia
by: Elisabetta Indelicato, et al.
Published: (2023-10-01)
by: Elisabetta Indelicato, et al.
Published: (2023-10-01)
Mechanism of action of 2-aminobenzamide HDAC inhibitors in reversing gene silencing in Friedreich’s ataxia
by: Elisabetta eSoragni, et al.
Published: (2015-03-01)
by: Elisabetta eSoragni, et al.
Published: (2015-03-01)
Reversible Axonal Dystrophy by Calcium Modulation in Frataxin-Deficient Sensory Neurons of YG8R Mice
by: Belén Mollá, et al.
Published: (2017-08-01)
by: Belén Mollá, et al.
Published: (2017-08-01)
Oxidative Stress and Antioxidant Therapies in Friedreich’s Ataxia
by: Félix Javier Jiménez-Jiménez, et al.
Published: (2025-09-01)
by: Félix Javier Jiménez-Jiménez, et al.
Published: (2025-09-01)
Therapeutic combination of L-ascorbic acid, N-acetylcysteine, and dimethyl fumarate in Friedreich’s ataxia: insights from in vitro models
by: Fred Jonathan Edzeamey, et al.
Published: (2025-12-01)
by: Fred Jonathan Edzeamey, et al.
Published: (2025-12-01)
Nrf2 Induction Re-establishes a Proper Neuronal Differentiation Program in Friedreich’s Ataxia Neural Stem Cells
by: Piergiorgio La Rosa, et al.
Published: (2019-07-01)
by: Piergiorgio La Rosa, et al.
Published: (2019-07-01)
Prolonged treatment with pimelic o-aminobenzamide HDAC inhibitors ameliorates the disease phenotype of a Friedreich ataxia mouse model
by: Chiranjeevi Sandi, et al.
Published: (2011-06-01)
by: Chiranjeevi Sandi, et al.
Published: (2011-06-01)
A Drug Combination Rescues Frataxin-Dependent Neural and Cardiac Pathophysiology in FA Models
by: Rosella Abeti, et al.
Published: (2022-05-01)
by: Rosella Abeti, et al.
Published: (2022-05-01)
Biophysical characterisation of the recombinant human frataxin precursor
by: Ignacio Hugo Castro, et al.
Published: (2018-03-01)
by: Ignacio Hugo Castro, et al.
Published: (2018-03-01)
AAV8 gene therapy reverses cardiac pathology and prevents early mortality in a mouse model of Friedreich’s ataxia
by: Joshua C. Chang, et al.
Published: (2024-03-01)
by: Joshua C. Chang, et al.
Published: (2024-03-01)
Editorial: The mechanistic investigation and emerging therapies for Friedreich’s ataxia
by: Yina Dong, et al.
Published: (2025-02-01)
by: Yina Dong, et al.
Published: (2025-02-01)
Molecular Defects in Friedreich’s Ataxia: Convergence of Oxidative Stress and Cytoskeletal Abnormalities
by: Frances M. Smith, et al.
Published: (2020-11-01)
by: Frances M. Smith, et al.
Published: (2020-11-01)
Friedreich’s Ataxia: A Neuronal Point of View on the Oxidative Stress Hypothesis
by: Barbara Carletti, et al.
Published: (2014-09-01)
by: Barbara Carletti, et al.
Published: (2014-09-01)
Acute frataxin knockdown in induced pluripotent stem cell-derived cardiomyocytes activates a type I interferon response
by: M. Grazia Cotticelli, et al.
Published: (2023-05-01)
by: M. Grazia Cotticelli, et al.
Published: (2023-05-01)
Drp1‐dependent peptide reverse mitochondrial fragmentation, a homeostatic response in Friedreich ataxia
by: Joseph Johnson, et al.
Published: (2021-05-01)
by: Joseph Johnson, et al.
Published: (2021-05-01)
Ferroptosis in Friedreich’s Ataxia: A Metal-Induced Neurodegenerative Disease
by: Piergiorgio La Rosa, et al.
Published: (2020-11-01)
by: Piergiorgio La Rosa, et al.
Published: (2020-11-01)
Resúmenes de los trabajos sobre la atrofia espinocerebelosa
by: Congreso Nacional de Neurolog'ia
Published: (2010-03-01)
by: Congreso Nacional de Neurolog'ia
Published: (2010-03-01)
Resúmenes de los trabajos sobre la atrofia espinocerebelosa
by: Congreso Nacional de Neurolog'ia
Published: (2010-03-01)
by: Congreso Nacional de Neurolog'ia
Published: (2010-03-01)
Antisense oligonucleotide therapy for patients with Friedreich’s ataxia carrying the c.165+5G>C splicing mutation
by: Pouiré Yameogo, et al.
Published: (2025-09-01)
by: Pouiré Yameogo, et al.
Published: (2025-09-01)
Frataxin is essential for zebrafish embryogenesis and pronephros formation
by: Wesley S. Ercanbrack, et al.
Published: (2024-12-01)
by: Wesley S. Ercanbrack, et al.
Published: (2024-12-01)
Anesthetic Management on a Patient with Friedreichs Ataxia
by: Ulku Ozgul, et al.
Published: (2013-12-01)
by: Ulku Ozgul, et al.
Published: (2013-12-01)
Molecular and Cellular Substrates for the Friedreich Ataxia. Significance of Contactin Expression and of Antioxidant Administration
by: Antonella Bizzoca, et al.
Published: (2020-09-01)
by: Antonella Bizzoca, et al.
Published: (2020-09-01)
Autosomal recessive ataxias: 20 types, and counting Ataxias autossômicas recessivas: 20 tipos e muito mais
by: Emília Katiane Embiruçu, et al.
Published: (2009-12-01)
by: Emília Katiane Embiruçu, et al.
Published: (2009-12-01)
Similar Items
-
Inducible and reversible phenotypes in a novel mouse model of Friedreich’s Ataxia
by: Vijayendran Chandran, et al.
Published: (2017-12-01) -
Neurobehavioral deficits of mice expressing a low level of G127V mutant frataxin
by: Daniel Fil, et al.
Published: (2023-02-01) -
Glial cell activation precedes neurodegeneration in the cerebellar cortex of the YG8–800 murine model of Friedreich ataxia
by: Andrés Vicente-Acosta, et al.
Published: (2024-10-01) -
Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency
by: Nicole M. Sayles, et al.
Published: (2023-10-01) -
Loss of Frataxin induces iron toxicity, sphingolipid synthesis, and Pdk1/Mef2 activation, leading to neurodegeneration
by: Kuchuan Chen, et al.
Published: (2016-06-01)
