Publications by Nicole Déglon

Dysfunction of homeostatic control of dopamine by astrocytes in the developing prefrontal cortex leads to cognitive impairments

Nicole Déglon, Graham Knott, Corrado Cali, Tamara Zehnder, Andrea Chicca, Luca Pucci

Astrocytes orchestrate neural development by powerfully coordinating synapse formation and function and, as such, may be critically involved in the pathogenesis of neurodevelopmental abnormalities and cognitive deficits commonly observed in psychiatric dis ...

2020

Scalable Production of AAV Vectors in Orbitally Shaken HEK293 Cells

Florian Maria Wurm, Nicole Déglon, Bernard Schneider, Vivianne Padrun, Gabriel Vachey, Daniel Roman Blessing

Adeno-associated virus (AAV) vectors are currently among the most commonly applied for in vivo gene therapy approaches. The evaluation of vectors during clinical development requires the production of considerable amounts of highly pure and potent vectors. ...

2019

Validation of Scalable and Compliant AAV Production

Florian Maria Wurm, Nicole Déglon, Bernard Schneider, Gabriel Vachey, Daniel Roman Blessing

Cell Press2017

Suspension-Adapted HEK 293 Cells in Orbital Shaken Bioreactors for the Production of Adeno-Associated Virus Vectors

Florian Maria Wurm, Nicole Déglon, Bernard Schneider, Daniel Roman Blessing

Nature Publishing Group2016

Transient Production of Recombinant Adeno-Associated Virus (AAV) Vectors for Gene Therapy Applications Using Suspension-Adapted HEK 293 Cells in Orbital Shaken Bioreactors

Florian Maria Wurm, Lucia Baldi Unser, Nicole Déglon, Bernard Schneider, Monique Montavon, Daniel Roman Blessing

Nature Publishing Group2015

Connexin 30 sets synaptic strength by controlling astroglial synapse invasion

Nicole Déglon, Graham Knott

Astrocytes play active roles in brain physiology by dynamic interactions with neurons. Connexin 30, one of the two main astroglial gap-junction subunits, is thought to be involved in behavioral and basic cognitive processes. However, the underlying cellula ...

Nature Publishing Group2014

Diminished hippocalcin expression in Huntington's disease brain does not account for increased striatal neuron vulnerability as assessed in primary neurons

Nicole Déglon, Ozgün Gökce, Nikita Rudinskiy

Hippocalcin is a neuronal calcium sensor protein previously implicated in regulating neuronal viability and plasticity. Hippocalcin is the most highly expressed neuronal calcium sensor in the medium spiny striatal output neurons that degenerate selectively ...

Wiley-Blackwell2009

Sustained Effects of Nonallele-Specific Huntingtin Silencing

Nicole Déglon

Objective: Huntington's disease (HD) is a fatal autosomal dominant neurodegenerative disorder caused by a polyglutamine expansion in the huntingtin (htt) protein. No cure is available to date to alleviate neurodegeneration. Recent studies have demonstrated ...

Wiley-Blackwell2009

Implication of the JNK pathway in a rat model of Huntington's disease

Patrick Aebischer, Nicole Déglon

Huntington's disease (HD) is a neurodegenerative disorder resulting from the expansion of a glutamine repeat (polyQ) in the N-terminus of the huntingtin (htt) protein. Expression of polyQ-containing proteins has been previously shown to induce various cell ...

2009

Striatal and nigral pathology in a lentiviral rat model of Machado-Joseph disease

Nicole Déglon

Machado-Joseph disease (MJD) is a fatal, dominant neurodegenerative disorder. MJD results from polyglutamine repeat expansion in the MJD-1 gene, conferring a toxic gain of function to the ataxin-3 protein. In this study, we aimed at overexpressing ataxin-3 ...

Oxford University Press2008

Haloperidol protects striatal neurons from dysfunction induced by mutated huntingtin in vivo

Nicole Déglon

Huntington's disease (HD) results from an abnormal polyglutamine extension in the N-terminal region of the huntingtin protein. This mutation causes preferential degeneration of striatal projection neurons. We previously demonstrated, in vitro, that dopamin ...

Elsevier2008

Effects of long-term and global huntingtin silencing

Nicole Déglon

Mary Ann Liebert2008

Dysregulation of gene expression in primary neuron models of Huntington's disease shows that polyglutamine-related effects on the striatal transcriptome may not be dependent on brain circuitry

Diana Zala, Patrick Aebischer, Valérie Gafner, Nicole Déglon, Heike Runne, Ozgün Gökce

Gene expression changes are a hallmark of the neuropathology of Huntington's disease (HD), but the exact molecular mechanisms of this effect remain uncertain. Here, we report that in vitro models of disease comprised of primary striatal neurons expressing ...

2008

Metabolic correction in oligodendrocytes derived from metachromatic leukodystrophy mouse model by using encapsulated recombinant myoblasts

Patrick Aebischer, Nicole Déglon

In an effort to develop an encapsulated cell-based system to deliver arylsulfatase A (ARSA) to the central nervous system of metachromatic leukodystrophy (MLD) patients, we engineered C2C12 mouse myoblasts with a retroviral vector containing a full-length ...

2007

Neuroprotection by Hsp104 and Hsp27 in lentiviral-based rat models of Huntington's disease

Patrick Aebischer, Valérie Gafner, Nicole Déglon

Huntington's disease (HD) is an inherited neurodegenerative disorder caused by an expansion of glutamine repeats in the huntingtin (htt) protein. Abnormal protein folding and the accumulation of mutated htt are hallmarks of HD neuropathology. Heat-shock pr ...

Elsevier2007

Expression of mutated huntingtin fragment in the putamen is sufficient to produce abnormal movement in non-human primates

Patrick Aebischer, Nicole Déglon

Huntington's disease (HD) is a neurological disorder characterized by striatal degeneration, motor symptoms and complex neuropsychiatric alterations. There is currently no genetic model of HD in non-human primates (NHPs). In this study we investigated neur ...

2007

Inhibition of calcineurin by FK506 protects against polyglutamine-huntingtin toxicity through an increase of huntingtin phosphorylation at S421

Patrick Aebischer, Nicole Déglon

Huntington's disease (HD) is caused by an abnormal expanded polyglutamine (polyQ) repeat in the huntingtin protein. Insulin-like growth factor-1 acting through the prosurvival kinase Akt mediates the phosphorylation of huntingtin at S421 and inhibits the t ...

2006

Lentivirus-mediated expression of glutathione peroxidase: neuroprotection in murine models of Parkinson's disease

Patrick Aebischer, Jean-Charles Bensadoun, Nicole Déglon

Reactive oxygen species are considered to contribute to the pathogenesis of Parkinson's disease (PD). In order to study viral vector-mediated overexpression of the antioxidant enzyme glutathione peroxidase (GPX) as a potential neuroprotective approach in b ...

2006

CA150 expression delays striatal cell death in overexpression and knock-in conditions for mutant huntingtin neurotoxicity

Diana Zala, Patrick Aebischer, Nicole Déglon

Transcriptional dysregulation caused by expanded polyglutamines (polyGlns) in huntingtin (htt) may be central to cell-autonomous mechanisms for neuronal cell death in Huntington's disease (HD) pathogenesis. We hypothesized that these mechanisms may involve ...

2006

Akt is altered in an animal model of Huntington's disease and in patients

Patrick Aebischer, Valérie Gafner, Nicole Déglon

The insulin-like growth factor I (IGF-1)/Akt pathway plays a crucial role in Huntington's disease by phosphorylating the causative protein, polyQ-huntingtin, and abolishing its toxic properties [Humbert et al. (2002)Dev. Cell, 2, 831-837; Rangone et al. (2 ...

2005