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BIOGRAPHICAL SKETCH BONNE, Gisèle,

biographical sketch bonne, gisèle, born 21/04/1966 senior researcher (dr1 inserm) deputy director, center of research
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BIOGRAPHICAL SKETCH

BONNE, Gisèle, born 21/04/1966
Senior researcher (DR1 Inserm)
Deputy Director, Center of Research in Myology (CRM),
PitiéSalpêtrière Hosp, Paris
EDUCATION/TRAINING
INSTITUTION AND LOCATION
DEGREE
YEAR(s)
FIELD OF STUDY
Denis Diderot University (Paris VII), Paris, France
DEA
1989
Developmental Physiology
Pierre et Marie Curie University (Paris VI), Paris
PhD
1994
Developmental Physiology
Colorado University at Boulder, CO, USA
Postdoctoral
19951996
Molecular Genetics
Pierre et Marie Curie University (Paris VI), Paris
HDR
2002
Molecular Genetics
A. Positions and Honours (selection)
19962005: Inserm Research Associate (CR: Chargé de recherche)
2005 : Inserm Research Director (DR: Directeur de recherche).
2014 : Deputy director & team leader. CRM. InsermUPMC U974, CNRS
FRE3617, AIM, Paris.
Honors: ENMC Research committee (20072014); Scientific Council
LefoulonDelalande Fondation (2009present); Executive Board World
Muscle Society (2010present); Vice chair (20122014) & Chair
(2015present) of French Society of Myology; Associated Editor of
Journal of Neuromuscular Diseases (2014present); Inserm Scientific
CouncilSection 3 (20162020).
Awards: BettencourtSchuller Foundation prize (1994); Translational
research contract Cardiogenetic service, PitiéSalpêtrière Hosp.
(20022014).
B. Publications 154 total, 24 first, 32 second last, 44 last, total
7971 citations, hindex 47
*
Bonne G, et al. Mutations in the gene encoding lamin A/C cause
autosomal dominant EmeryDreifuss dystrophy. Nature Genet, 1999,
21:285288. Arimura T…Bonne G. Mouse model carrying H222PLmna
mutation develops muscular dystrophy and dilated cardiomyopathy
similar to human striated muscle laminopathies. Hum Mol Genet.
2005;14:15569. Meune C…Bonne G, Pinto YM, Duboc D. Primary
prevention of sudden death in patients with lamin A/C gene
mutations N Engl J Med. 2006; 354:20910. Muchir A…Bonne G, Worman
HJ. Treatment with selumetinib preserves cardiac function and
improves survival in cardiomyopathy caused by mutation in the
lamin A/C gene. Cardiovasc Res. 2012; 93:3119. Cattin ME…Bonne G.
Heterozygous LmnadelK32 mice develop dilated cardiomyopathy
through a combined pathomechanism of haploinsufficiency and
peptide toxicity. Hum Mol Genet. 2013; 22:315264. Perovanovic …,
Bonne G, Hoffman EP. Laminopathies disrupt epigenomic
developmental programs and cell fate. Sci Transl Med.
2016;8(335):335ra58.
*
Non scientific publications (selection) Bonne et al.
EmeryDreifuss Muscular Dystrophy. GeneReviews™ (www.ncbi.nlm.nih.gov.gate2.inist.fr/books/NBK1436/).
Bonne G, Gene therapy approaches for Laminopathies. (http://youtu.be/vjUoYom1220).
C. Research Support (over the last 10 years)
DFG/UPMC: MYOGRAD, International Research Training Group for Myology
(PI S Spuler (Germany)/T Voit (France), 2010 2014) 20 PhD
Fellowships among which 3 are available to the Bonne lab.
CURECMD: Gene therapy of LCMD (PI G Bonne, 2012 2015); 120 000 US
$ available to the Bonne Lab.
CURECMD: Conduction defect mechanisms in LCMD (PI A Muchir,
20142015) 30 000 US $ to Bonne Lab.
France Genomics: MyoCapture Consortium: novel genes for myopathies
(1000 exomes available for the consortium, PI J Laporte, 20132016).
300 exomes available to the Bonne lab.
Association Institut de Myologie, U974 Strategic Grant to G Bonne
Team (PI: G Bonne, 2016): 566 000 €.
CURECMD/Marcio A Fundation: Gene therapy of LCMD (PI G Bonne,
2016 2017): 100 000 € +40 000 U S$.
ERACVD: Novel RNA based therapies for treatmentresistant forms of
LMNAcardiomyopathy (PI Y Pinto, 3 team consortium, 20172020): 180
440 € available to Bonne lab.
D. Activities on knowledge management
Coordinator of French network on "EDMD & other nuclear envelope
diseases" (2000 ).
EU cocoordination: EUROMEN (FP5, 20002003), EuroLaminopathies
(FP6, 20062009), NMDCHIPS (FP7, 20082011).
Coordinator of international Human Frontier Science Program network
on laminopathies (20032005).
Cocoordinator Inserm/JSPS: Pathophysiological & preclinical therapy
studies of hereditary cardiomyopathies (20092011).
Coordinator of the Myology Institute teams within the National
Consortium “MyoCapture” (20132016).

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