Noëlle Dufour

Commissariat à l'Énergie Atomique et aux Énergies Alternatives — France

Neuroscience · Life Sciences

24h-index3.2kcitations59works2.02yr avg
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Research Topics

Genetic Neurodegenerative Diseases(30), Mitochondrial Function and Pathology(18), Virus-based gene therapy research(10), Neurological disorders and treatments(10), Parkinson's Disease Mechanisms and Treatments(6)

Publications59 total

Translational importance of non-human primates for the study of the immune response in gene therapy procedures involving in vivo administration of rAAV vectors
Revue de primatologie·2025Open Access
Corrigendum to “Huntingtin lowering impairs the maturation and synchronized synaptic activity of human cortical neuronal networks derived from induced pluripotent stem cells” [Neurobiology of Disease Volume 200 (2024, Oct 1st) - Article# YNBDI_106630]
Neurobiology of Disease·2024· 1 citedOpen Access
I011 Functional impacts of Huntingtin lowering on the synaptic activity of neuron-glia networks derived from human induced pluripotent stem cells
2024Open Access
Huntingtin lowering impairs the maturation and synchronized synaptic activity of human cortical neuronal networks derived from induced pluripotent stem cells
Neurobiology of Disease·2024· 5 citedOpen Access
Mono- and Biallelic Inactivation of Huntingtin Gene in Patient-Specific Induced Pluripotent Stem Cells Reveal HTT Roles in Striatal Development and Neuronal Functions
Journal of Huntington s Disease·2024· 4 citedOpen Access
Reactive astrocytes promote proteostasis in Huntington’s disease through the JAK2-STAT3 pathway
Brain·2022· 63 citedOpen Access
The C-Terminal Domain of LRRK2 with the G2019S Substitution Increases Mutant A53T α-Synuclein Toxicity in Dopaminergic Neurons In Vivo
International Journal of Molecular Sciences·2021· 10 citedOpen Access
Reactive astrocytes promote proteostasis in Huntington’s disease through the JAK2-STAT3 pathway
2021· 8 citedOpen Access
The C-terminal fragment of LRRK2 with the G2019S substitution increases the neurotoxicity of mutant A53T α-synuclein in dopaminergic neurons in vivo
Research Square (Research Square)·2020Open Access
The C-terminal fragment of LRRK2 with the G2019S substitution increases the neurotoxicity of mutant A53T α-synuclein in dopaminergic neurons in vivo
Research Square (Research Square)·2020Open Access
The C-terminal domain of LRRK2 with the G2019S mutation is sufficient to produce neurodegeneration of dopaminergic neurons in vivo
Neurobiology of Disease·2019· 21 citedOpen Access
A42 Reactive astrocytes promote proteostasis in huntington’s disease
2018· 3 cited
The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin
Brain·2018· 43 citedOpen Access
Preclinical Evaluation of a Lentiviral Vector for Huntingtin Silencing
Molecular Therapy — Methods & Clinical Development·2017· 15 citedOpen Access
Characterization of the striatal kinase Dclk3 and its neuroprotective effects againstmutant huntingtin
HAL (Le Centre pour la Communication Scientifique Directe)·2015
Gene transfer engineering for astrocyte-specific silencing in the CNS
Gene Therapy·2015· 27 citedOpen Access
541. Increased Integration of Lentiviral Vector Carrying a D167H Integrase Relies on the Presence of LEDGF/p75
Molecular Therapy·2015Open Access
The striatal long noncoding RNA Abhd11os is neuroprotective against an N-terminal fragment of mutant huntingtin in vivo
Neurobiology of Aging·2014· 52 citedOpen Access
Loss of the thyroid hormone-binding protein Crym renders striatal neurons more vulnerable to mutant huntingtin in Huntington's disease
Human Molecular Genetics·2014· 31 citedOpen Access
Renal Tolerance to Stereotactic Body Radiation Therapy: An Assessment of Volume and Anatomical Site Irradiated
International Journal of Radiation Oncology*Biology*Physics·2014· 2 citedOpen Access
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Frequent Co-authors

Nicole Déglon(34), Philippe Hantraye(28), Emmanuel Brouillet(27), Martine Guillermier(16), Gilles Bonvento(16), Gwennaëlle Aurégan(15), Marie‐Claude Gaillard(14), Alexis‐Pierre Bemelmans(13), Fanny Petit(13), Raymonde Hässig(12), Sandro Alves(7), Laurie Galvan(7), Carole Escartin(7), Karine Cambon(7), Anselme L. Perrier(7), Gwenaëlle Aurégan(7), Jean‐Marc Elalouf(6), Diane Houitte(6), Michel de Chaldée(6), Frédéric Saudou(5)