| Guy Lenaers |
 |
Function
: CR CNRS, Group Leader : inherited optic neuropathies
and mitochondrial disorders
Academic and research
track :
I graduate in 1985 in Toulouse from the DEA Biologie et
Santé (Université Paul Sabatier), then went
to the marine station of Banyuls sur Mer to study dinoflagelatte
phylogeny in Luc Maroteaux, Marie-Odile Soyer-Gobillard and
Michel Herzog group. I read my thesis in 1990, at Université Montpellier
II and went as a post-doctorate in Paul Russell lab at Scripps
Clinic Research Institute (San Diego, CA, USA) to study fission
yeast cell cycle regulation by tyrosine phosphatases. In
1992, I was recruited by the CNRS and joined André Picard
group in Banyuls sur Mer, to study fission yeast gene expression
along the cell cycle. In 1999, I moved to Bernard Ducommun
laboratory in Toulouse and started to study mitochondrial
dynamic, uncovering OPA1 and its involvement in Dominant
Optic Atrophy. In 2003, I obtained my « Habilitation à Diriger
les Recherches » from the Université de
Toulouse. In 2004, I started my group in Christian Hamel
department at the “Institut des Neurosciences de Montpellier”.
Main achievements
:
• During my post doctoral fellowship, I uncovered
the role of the tyrosine phosphatases cdc25 and pyp3 in the
control of the G2/M transition of the cell cycle.
• In 2000, I contributed to show that the mitochondrial dynamin-related
protein, OPA1, is responsible for the majority of cases of Kjer disease, one
of the leading cause of inherited optic neuropathy.
• Recently, we identified a novel gene OPA7 involved in a form of syndromic
Dominant Optic Atrophy.
Research interests
:
• Discovering the genes causing inherited diseases
of the optic nerve, for their extraordinary potential to
improve our understanding of the mitochondrial physiology
and pathophysiology in the optic nerve function.
• Studying the the natural history of inherited optic neuropathies and
understanding why retinal ganglion cells are susceptible to certain mitochondrial
defects and how we can act on mitochondrial metabolism to restore their functional
integrity.
• Understanding the roles of OPA genes in the dynamic and structure of
the mitochondrial inner membrane that support the respiratory complexes.
• Using this knowledge to provide accurate genetic counselling and design
neuroprotective strategies to treat patients and prevent low vision and blindness.
Selected publications
:
Original publications :
Olichon A, Elachouri G, Baricault L, Delettre C, Belenguer
P, Lenaers G.
OPA1 alternate splicing uncouples an evolutionary conserved
function in mitochondrial fusion from a vertebrate restricted
function in apoptosis.
Cell Death Differ. 2007 Apr;14(4):682-92.
Reynier P, Amati-Bonneau P, Verny C, Olichon A, Simard G,
Guichet A, Bonnemains C, Malecaze F, Malinge MC, Pelletier
JB, Calvas P, Dollfus H, Belenguer P, Malthiery Y, Lenaers
G, Bonneau D.
OPA3 gene mutations responsible for autosomal dominant optic
atrophy and cataract.
J Med Genet. 2004 Sep;41(9):e110.
Olichon A, Baricault L, Gas N, Guillou E, Valette A, Belenguer
P, Lenaers G.
Loss of OPA1 perturbates the mitochondrial inner membrane
structure and integrity, leading to cytochrome c release
and apoptosis.
J Biol Chem. 2003 Mar 7;278(10):7743-6.
Lenaers G, Delettre C, Griffoin JM, Gigarel
N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel
C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B,
Ducommun B, Kaplan J, Hamel CP.
Nuclear gene OPA1, encoding a mitochondrial dynamin-related
protein, is mutated in dominant optic atrophy.
Nat Genet. 2000 Oct;26(2):207-10.
Reviews :
Delettre C, Hamel CP, Lenaers G.
Optic Atrophy, Type I.
http://www.genetests.org/servlet/access?id=&key=&fcn=y&fw=Nif0&filename=/profiles/opa/index.html.
NIH • University
of Washington, Seattle, July 2007.
Hamel CP, Lenaers G.
Les neuropathies optiques héréditaires
Encyclopédie Médicale Continue 21-480-E-30,
Juillet 2007.
Olichon A, Guillou E, Delettre C, Landes T, Arnaune-Pelloquin
L, Emorine LJ, Mils V, Daloyau M, Hamel C, Amati-Bonneau
P, Bonneau D, Reynier P, Lenaers G, Belenguer
P.
Mitochondrial dynamics and disease, OPA1.
Biochim Biophys Acta. 2006 May-Jun;1763(5-6):500-9.
Other fonctions :
Membre of Médecins Sans Frontières:
Head
of a tuberculosis diagnosis laboratory in the Afar Region,
Ethiopia (dec 2003- may 2004).
Membre of Comité de Secours Internationaux :
Head
of a medical rescue team in Pakistan Cachmira earthquake (oct 2005).
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