Use of induced pluripotent stem cells to explore molecular mechanisms of accelerated aging disorders
2013.3.11
Xavier Nissan I-STEM, France
March 11th 2013, 12h00, Biocant (Cantanhede)

Host: Lino Ferreira


Synopsis:
Xavier Nissan joined I-Stem* in 2004 with the mission to develop and manage several scientific projects focusing on microRNAs in human development. In 2007, he started a PhD and developed methods permitting the derivation of homogeneous and functional populations of keratinocytes and melanocytes from pluripotent stem cells. In 2010, Dr Xavier Nissan successfully initiated a research program on Hutchinson- Gilford Progeria Syndrome (HGPS). He is now leading the team “Stem cells and premature aging disorders” of I-Stem and is member of the scientific board of the institute.

Progeria, also known as Hutchinson-Gilford Progeria Syndrome (HGPS), is a rare, fatal genetic disease characterized by an appearance of accelerated aging in children. This syndrome is typically caused by mutations in codon 608 (G608G) of the LMNA leading to the production of a mutated form of Lamin A precursor called Progerin. In HGPS, Progerin accumulates in cells causing progressive molecular defects including nuclear shape abnormalities, chromatin disorganization, DNA damages and delay in cell proliferation. Although two clinical trials have recently produced promising results, as well as in vitro and in vivo, there is currently no cure for HGPS patients. In collaboration with Pr Nicolas Lévy, our team has addressed this challenge by using the unique potential of pluripotent stem cells. Thanks to this unlimited and standardized biological resource we have first, developed cellular tools to study molecular mechanisms involved in this pathology and second, used the iPS cells technology to identify new therapeutic drugs.

*Created in 2004, I-Stem results from the combination of two different and independent entities: a joined Research Unit of INSERM (UMR-861 unit), the University Evry-Val d’Essonne and the laboratory managed by CECS (Centre pour l’Etude des Cellules Souches). Financially supported by the French Muscular Disease Association (AFM), I-Stem integrates three different activities: a basic biological research, a technological research for development and application, and a biological resource center. The main objective of I-Stem is to explore the therapeutic potential of pluripotent stem cells in order to propose to patients therapeutic products applied to monogenic diseases. I-Stem is an uncontested worldwide leader in the field of pluripotent stem cells conducing drug screenings for rare diseases and cell therapy clinical trials.
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