Pascale Bomont
  • E-mail :[email]
  • Phone : +33 4 99 63 60 73
  • Location : Montpellier, France
Last update 2012-03-09 21:36:10.735

Pascale Bomont PhD Neurosciences

Course and current status

Present position (from 09/2011)

AVENIR-INSERM group leader at INM (INSERM U1051); Montpellier, France

Education

2010 H.D.R (Habilitation to direct Research); Aix-Marseille II University, France

2002 PhD in Molecular and Cellular Biology; University Louis Pasteur, Strasbourg, France

Research experience/positions

2011 Senior INSERM Researcher (CR1)

2007 Junior INSERM Researcher (CR2)

2007-2011 INSERM Researcher in Neurobiology; INMED; Marseille, France (Dir. Pr A Represa)

2003-2007 Postoctoral Fellow in Neurobiology/Cell Biology; LICR; San DIego, USA (Dir. Pr DW Cleveland)

1998-2002 PhD in Human Genetics; IGBMC; Strasbourg, France (Dir. Pr JL Mandel)

Scientific summary

The Ubiquitin Proteasome System (UPS) is impaired in many neurodegenerative disorders and its role in neurodegeneration is the focus of intense scrutiny. However, solving its implication of in neuronal death represents a considerable challenge and systems in which neurodegeneration is directly caused by mutations in component(s) of the UPS are very valuable models for this aim.

The identification of gigaxonin, the substrate adaptor of a Cul3-E3 ubiquitin ligase, as the defective protein in the fatale neurodegenerative disorder Giant Axonal Neuropathy (GAN), prompted us to choose this model as a direct way to study the role of the UPS in neurodegeneration. The broad degeneration of the nervous system together with the generalized disorganization of Intermediate Filaments in patients, point to a key role for gigaxonin in sustaining both neuronal survival and cytoskeleton architecture.

Our group is dedicated to deciphering the pathophysiology of GAN but also to developing a specific diagnostic test and the first therapeutic approaches for this fatale disease. We are studying the pathways of cell death and cytoskeleton dynamics in new neuronal models that are also used as readout tests to evaluate the efficacy of therapeutic vehicules generated by our collaborators. We are using multidisciplinary approaches encompassing human genetics, cell biology, biochemistry and animal models.

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