Home

Person

Nicole Déglon

About
Privacy
Disclaimer

Graph Chatbot

Related publications (59)

Page 2 of 3

Long-term lentiviral-mediated expression of ciliary neurotrophic factor in the striatum of Huntington's disease transgenic mice

Patrick Aebischer, Nicole Déglon, Jean-Charles Bensadoun, Diana Zala

Ciliary neurotrophic factor (CNTF) has been shown to prevent behavioral deficits and striatal degeneration in neurotoxic models of Huntington's disease (HD), but its effect in a genetic model has not been evaluated. Lentiviral vectors expressing the human ...

2004

Delivery of erythropoietin by encapsulated myoblasts in a genetic model of severe anemia

Patrick Aebischer, Bernard Schneider, Nicole Déglon

BACKGROUND: Existing animal models of anemia inadequately reflect the hematocrit usually present in chronic renal failure (CRF) patients and do not permit long-term treatment studies. The transgenic mouse strain 134.3LC (Epo-TAg(H)) displays a severe chron ...

2002

Lentivirally delivered glial cell line-derived neurotrophic factor increases the number of striatal dopaminergic neurons in primate models of nigrostriatal degeneration

Patrick Aebischer, Nicole Déglon

The primate striatum contains tyrosine hydroxylase (TH)-immunoreactive (ir) neurons, the numbers of which are augmented after dopamine depletion. Glial cell line-derived neurotrophic factor (GDNF) strongly modulates the viability and phenotypic expression ...

2002

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

Lentiviral-mediated gene transfer to model triplet repeat disorders

Patrick Aebischer, Nicole Déglon, Diana Zala

This chapter describes the potential use of viral-mediated gene transfer in the central nervous system as a new strategy in developing animal models of neurodegenerative diseases. To illustrate the approach, procedures for the production of lentiviral vect ...

2004

Complete and long-term rescue of lesioned adult motoneurons by lentiviral-mediated expression of glial cell line-derived neurotrophic factor in the facial nucleus

Patrick Aebischer, Nicole Déglon

To date, delivery of neurotrophic factors has only allowed to transiently protect axotomized facial motoneurons against cell death. In the present report, long-term protection of these neurons was evaluated by continuously expressing the neurotrophic facto ...

2000

Survival of encapsulated human primary fibroblasts and erythropoietin expression under xenogeneic conditions

Patrick Aebischer, Bernard Schneider, Nicole Déglon

Allogeneic cells are the most attractive source for cell transplantation, as the use of xenogeneic cells is hampered by safety concerns and the use of autologous cells involves practical difficulties. The immune rejection of allogeneic cells can be overcom ...

2004

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

Graham Knott, Nicole Déglon, Corrado Cali

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

Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment

Patrick Aebischer, Nicole Déglon, Diana Zala, Valérie Gafner

A lentiviral vector expressing a mutant huntingtin protein (htt171-82Q) was used to generate a chronic model of Huntington's disease (HD) in rat primary striatal cultures. In this model, the majority of neurons expressed the transgene so that Western blot ...

2005

Viral vector-mediated gene therapy for Parkinson's disease

Patrick Aebischer, Nicole Déglon

Parkinson's disease (PD) is a progressive neurodegenerative disorder that affects 1% of the population above the age of 60. The cause of the disease remains unknown. The histopathological hallmarks of the disease are intracytoplasmic Lewy bodies and dopami ...

2001

Rescue of motoneurons from axotomy-induced cell death by polymer encapsulated cells genetically engineered to release CNTF

Patrick Aebischer, Nicole Déglon

The neurodegenerative disease amyotrophic lateral sclerosis (ALS) results from the progressive loss of motoneurons, leading to death in a few years. Ciliary neurotrophic factor (CNTF), which decreases naturally occurring and axotomy-induced cell death, may ...

1996

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

Patrick Aebischer, Nicole Déglon, Valérie Gafner

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

Scalable Production of AAV Vectors in Orbitally Shaken HEK293 Cells

Vivianne Padrun, Florian Maria Wurm, Bernard Schneider, Nicole Déglon

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

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

Connexin 30 sets synaptic strength by controlling astroglial synapse invasion

Graham Knott, Nicole Déglon

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

Efficient gene transfer and expression of biologically active glial cell line-derived neurotrophic factor in rat motoneurons transduced wit lentiviral vectors

Patrick Aebischer, Nicole Déglon

Several studies have shown the ability of human immunodeficiency virus type 1 (HIV1)-based lentiviral vectors to infect nondividing brain and retinal neurons with high efficiency and long-term expression of the transduced gene. We show that purified embryo ...

Wiley-Blackwell2000

Validation of Scalable and Compliant AAV Production

Florian Maria Wurm, Bernard Schneider, Nicole Déglon

Cell Press2017

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

A gene therapy approach to regulated delivery of erythropoietin as a function of oxygen tension

Patrick Aebischer, Nicole Déglon

Current therapy for several forms of anemia involves a weekly regime of multiple subcutaneous injections of recombinant human erythropoietin (hEpo). In an effort to provide a physiologically regulated administration of erythropoietin, we are developing cel ...

1997

Gene therapy for amyotrophic lateral sclerosis (ALS) using a polymer encapsulated xenogenic cell line engineered to secrete hCNTF

Patrick Aebischer, Frédéric Kaplan, Nicole Déglon

The gene therapy approach presented in this protocol employs a polymer encapsulated, xenogenic, transfected cell line to release human ciliary neurotrophic factor (hCNTF) for the treatment of Amyotrophic Lateral Sclerosis (ALS). A tethered device, containi ...

1996