Gisèle Bonne, PhD




MyoGrad Speaker, Paris
MyoGrad Subproject Speaker, Paris

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Phone: +33 1 42 16 57 23
Fax:     +33 1 42 16 57 00


Center of Research in Myology
UPMC - Inserm UMRS 974, CNRS FRE3617
Institut de Myologie
G.H. Pitié-Salpêtrière


Fields of responsibilty
MyoGrad Speaker, Paris – International Research Training Group for Myology Berlin-Paris
MyoGrad – Project 7: Structural elements of muscle

Scientific Background

1989-1994
INSERM UR 75, Faculté de Médecine Necker-Enfants Malades, Paris, France (Pr. J.P.
LEROUX) : The human cytochrome c oxidase complex: Study of its expression in foetus and adult and in pathologies associated with abnormalities of the mitochondrial genome (PhD)

1994 -1996
INSERM (French National Institute of Heath and Medical Research) Research Unit 153, GH La Pitié-Salpétrière, Paris, France (Dr. K. SCHWARTZ): Molecular genetics of familial
hypertrophic cardiomyopathy.

1995-1996
Department of Molecular, Cellular and Developmental Biology, University of Colorado,
Boulder, CO, USA (Dr. L. LEINWAND): Analyses of structural consequences of MYBPC3
mutations identified in familial hypertrophy cardiomyopathy.

since 1996
Research Director at INSERM (Permanent position) at INSERM-UPMC UMR974 (Director: Pr. Thomas Voit); Team leader: Genetics and Pathophysiology of neuromuscular disorders

Degrees

1986 Technician degree, option: Biochemical and biological analyses, Dijon.
1986 Cell culture technician degree, Dijon
1987-1988 Master of Science, option: Biochemistry, Paris.
1989 Diplôme d'Etudes Approfondies, physiology of development, Paris.
1994 PhD degree of physiology of the development, Paris.
1999 Habilitation for animal experimentation, National Veterinary school of Alfort.
2002 Habilitation to direct research (HDR)

Coordination of multi-center projects

2000-2003
EUROMEN: EUROpean Muscle Envelope Nucleopathies: application of molecular geneticadvances for the understanding and management of Emery-Dreifuss muscular dystrophies and associated skeletal and cardiac phenotypes (European Community): coordination of 11 clinical and genetical European teams

2001 – present
«French network of Emery-Dreifuss muscular dystrophies and other nucleopathies
(INSERM/AFM): coordination of 30 clinical and genetical French teams

2002-2005
Nuclear Envelope and muscle Disease, Human Frontier Science Program Organisation (HFSPO) en collaboration avec H. Worman (Columbia Universtity, New York, USA), P.nCollas (Oslo University, Norway), Yukiko Hayashi (National Institute of Neuroscience, Tokyo, Japan).

2006-2009
TNFLAMINAC: Glutathione and TNF in Emery-Dreifuss muscular dystrophy and other
laminopathies affecting striated muscles. N-acetylcysteine as a potential therapy (French National research Agency for rare disorders - ANR): coordination of 3 teams
2006-2009
Euro-Laminopathies: European network (EU-FP6): coordination among 10 participating
teams of the three Inserm teams composed of clinical genetic teams

Memberships

1996 – present
Groupe Cœur de l'Association Française contre les Myopathies
1997 – present
International Society for Heart Research (ISHR)
1998 – present
Groupe de Réflexion sur la Recherche Cardiovasculaire (GRRC) :
2000 – present
World Muscle Society: 2000
2002 – present
Board member of the Société Française de Myologie; Secretary since 2007
2003 – 2010
Member of the scientific council of Association Française contre les Myopathies (AFM)
2007 – present
Member of the editorial board of Neuromuscular Disorders
2007 – present
Member of the ENMC Research Committee (European NeuroMuscular Center)
2013 – present
Speaker of International Research Training Group for Myology - MyoGrad
2013 – present
Vice Chair of the French Myology Society
2013 – present
Member of the International Society of Heart Research, European Section


Awards and Honors

Award from Bettencourt-Schuller Foundation, 1994.


Selected publications


Bonne G, Carrier L, Bercovici J, Cruaud C, Richard P, Hainque B, Gautel M, Labeit S, James M, Beckman J, Weissenbach J, Vosberg HP, Fiszman M, Komajda M, Schwartz K. (1995) Cardiac myosin binding protein-C gene splice acceptor site mutation is associated with familial hypertrophic cardiomyopathy. Nature Genet. 11:438-440.

Bonne G, Di Barletta MR, Varnous S, Bécane HM, Hammouda EH, Merlini L, Muntoni F, Greenberg CR, Gary F, Urtizberea JA, Duboc D, Fardeau M, Toniolo D, Schwartz K. (1999) Mutations in the gene encoding lamin A/C cause autosomal dominant Emery-Dreifuss dystrophy. Nature Genet. 21:285-288.

Meune C, van Berlo JH, Anselme F, Bonne G, Pinto YM, Duboc D. (2006) Primary Prevention of Sudden death in patients with Lamins A/C gene mutations. NEJM. 354(2):209-210.

Muchir A, Pavlidis P, Decostre V, Herron AJ, Arimura T, Bonne G, Worman HJ. (2007) Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery- Dreifuss muscular dystrophy. J Clin Invest. 117(5):1282-1293.

Gueneau L, Bertrand AT, Jais JP, Salih MA, Stojkovic T, Wehnert M, Hoeltzenbein M, Spuler S, Saitoh S, Verschueren A, Tranchant C, Beuvin M, Lacene E, Romero NB, Heath S, Zelenika D, Voit T, Eymard B, Ben Yaou R, Bonne G. (2009) Mutations of the FHL1 gene cause Emery-Dreifuss muscular dystrophy. Am J Hum Genet. 85(3):338-53. Supervision of young researchers.