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French
Department of Neurobiology and Genetics

Team leader : Michel KOENIG

Contact : mkoenig@igbmc.u-strasbg.fr
Tél. +33 (0) 3 88 65 33 99

Research interests :

Understand the molecular mechanisms underlying recessive ataxias, through the identification of the defective genes by positional cloning and the subsequent use of animal and cellular models in order to understand the physiopathology, and to propose and test new therapeutic approaches.

Friedreich’s ataxia and other recessive ataxias
Team Members :

Permanent scientist(s) :
Michel KOENIG
Christine TRANCHANT

Post-doctoral fellow
Aline BENNASROUNE

Graduate Student(s):
Mathieu ANHEIM
Mirna ASSOUM
Dorra H'MIDA

Engineers/Technicians
Nathalie DROUOT
Laurence REUTENAUER

Main publications :

M.-C. Moreira, S. Klur, M. Watanabe, A. H. Németh, I. Le Ber, J.-C. Moniz, C. Tranchant, P. Aubourg, M. Tazir, L. Schöls, M. Pandolfo, J. B. Schulz, J. Pouget, P. Calvas, M. Shizuka-Ikeda, M. Shoji, M. Tanaka, L. Izatt, C. E. Shaw, E. Dunne, P. Bomont, T. Benhassine, N. Bouslam, A. Brice, J. Guimãraes, P. Mendonça, C. Barbot, P. Coutinho, J. Sequeiros, A. Dürr, J.-M. Warter, M. Koenig ((2004) ) Senataxin, the ortholog of a yeast RNA helicase, is mutant in ataxia-ocular apraxia 2 Nat. Genet 36 : 225-227

Simon, D., Seznec, H., Gansmuller, A., Carelle, N., Weber, P., Metzger, D., Rustin, P., Koenig, M., and Puccio, H. (2004), New FRDA mouse models with progressive cerebellar and sensory ataxia reveal an autophagic neurodegeneration in DRG. J. Neurosciences 24 (8) : 1987-1995

H. Seznec; D. Simon; L. Monassier; P. Criqui-Filip; A. Gansmuller; P. Rustin; M. Koenig and H. Puccio. Idebone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia. Hum Mol Genet 13: 1017-24

M.-C. Moreira, C. Barbot, N. Tachi, N. Kozuka, E. Uchida, T. Gibson, P. Mendonça, M. Costa, J. Barros, T Yanagisawa, M. Watanabe, Y. Ikeda, M Aoki, T Nagata, P. Coutinho, J. Sequeiros and M. Koenig (2001). The gene mutated in ataxia-ocular apraxia 1 (AOA1) encodes aprataxin, a new HIT/Zn-finger protein potentially involved in DNA repair. Nat. Genet. 29: 189-193

P. Bomont, L. Cavalier, F. Blondeau, C. Ben Hamida, S. Belal, M. Tazir, E. Demir, H. Topaloglu, R. Korinthenberg, B. Tüysüz, P. Landrieu, F. Hentati and M. Koenig (2000). The gene encoding gigaxonin, a new member of the cytoskeletal BTB/Kelch repeat family, is mutated in giant axonal neuropathy. Nat. Genet. 26: 370-374.