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Mental retardation: from genes to synapses, functions and dysfunctions

Roscoff (Brittany), France, October 7-11, 2010

 

Deadline for application: June 20, 2010

 

Chairperson: Barbara Bardoni

Institut de Pharmacologie Moléculaire et Cellulaire, CNRS UMR6097, Université de Nice Sophia-Antipolis,
660, Route des Lucioles, 06560 Valbonne, France
Phone: + 33 4 93957766/62 – Fax: + 33 4 93957708
Mail : bardoni@ipmc.cnrs.fr

 

Vice-Chairperson: Maria Vincenza Catania

Institute of Neurological Sciences, National Research Council (CNR),
Via Paolo Gaifami N° 18,  95126 Catania, Italie
Phone: + 39 095 7338111 /7338134 – Fa: + 39 095 7338110
Mail : m.catania@isn.cnr.it

 

In all studied populations, mental retardation (MR) is among the most common form of genetic handicaps with a prevalence of 1 to 3 % and constitutes a major medical and social problem. The causes of MR are extremely heterogeneous, ranging from environmental to genetic and even combinations of the two. Among the genetic causes of MR are chromosomal aberrations, cryptic subtelomeric deletions and mutations affecting single genes. MR can be divided into two classes: nonsyndromic and syndromic. In the syndromic form, the mental handicap is accompanied by a recognizable pattern of somatic, neurological, behavioural or metabolic abnormalities. Approximately 10-15% of about 1000 hereditary diseases associated with MR show an X-linked mode of inheritance (XLRM).

It is clear that the purpose of each scientist working in the field is the possibility to find a curative treatment to these disorders by the identification of the cellular defects induced by the gene mutation. A clue to find treatments comes from the identification of the mechanism of action of different gene products. Cloned genes involved in MR encode for proteins involved in different pathways of the biology of the cell and for most of these proteins we have very few information, in particular concerning their synaptic function. During recent years new technologies including CGH microarray (Comparative Genomic Hybridization) and high-throughput sequencing allowed the identification of a large number of genes involved in learning disabilities. For example, in 2000 about 33 X-linked-associated genes responsible MR have been identified, in 2003 at least 43 were recorded and today they are more than 90 and we can predict that the number will be easily and rapidly increased.

Starting from these considerations, we propose a Jacques Monod Conference entitled "Mental Retardation: from genes to synapses, functions and dysfunction" during which geneticists involved in the search for MR genes, and neuroscientists involved in understanding structure and mechanisms of function of synapses as well as other aspects of neuronal functions potentially relevant for MR, might exchange information and propose alternative research strategies. While genetics is essential to identify the cause of MR and address the pathogenesis, it is important to focus on neurobiology in order to make progress in the direction of treatment, matching medical relevance and basic science.

The conference will focus on the following topics:

  • Genetic and molecular bases of MR.
  • Animal models for cognitive deficit.
  • Structures and plasticity of synapses:
    • receptors trafficking;
    • synaptic cytoskeleton;
    • translational control at the synaptic level (pathways involved and RNA-binding proteins).

 

Invited speakers

(provisional titles)

 

BEN-ARI Yezekiel (Marseille, France)
Neuroarcheology: arguments in favour of an early presymptomatic expression of many neurological disorders

BARDONI Barbara (Valbonne, France)
RNA metabolism and learning disability

CATANIA Maria Vincenza (Catani, Italie)
 Group I metabotropic glutamate receptors: a role in neurodevelopmental disorders?

CHELLY Jamel (Paris, France)
Mutations in the β-tubulin gene TUBB2B result in asymmetrical polycrogyria

CHOQUET Daniel (Bordeaux, France)
Nanoscale Dynamic Organization of the Synapse 

COLLEAUX Laurence (Paris, France)
Unravelling the molecular and pathophysiological bases of autosomal recessive non-syndromic mental retardation. 

DIERSSEN Mara (Barcelona, Spain)
Decoding the genetics of Down syndrome 

FAGNI Laurent (Montpellier, France)
How can Shank3 induce spine dysgenesis and mental retardation?

FERGUSON Shawn (New Haven, USA)
Elucidating specialized mechanisms of membrane traffic in neurons

FERRAGUTI Francesco (Innsbruck, Austria)
Changes in the subcellular distribution of neurotransmitter receptors in mouse models of impaired learning 

FRANCIS Fiona (Paris, France)
Doublecortin knockout mice, hippocampal dysfunction and cognitive deficits

GALLI Thierry (Paris, France)
Role of exocytosis in neuronal differentiation.

GÉCZ Jozef (Adelaide, Australia)
Non-sense mediated mRNA decay (NMD) surveillance and intellectual disability

GIANGRANDE Angela (Illkirch, France)
The FMRP pathway in Drosophila melanogaster nervous system

HÜTTELMAIER Stefan (Halle, Germany
Subcellular RNA localization and Mental Retardation

KOSIK Ken (Santa Barbara, USA)
The Role of microRNAs in Synaptic Plasticity

MALINOW Roberto (San Diego, USA)
Synaptic AMPA receptor plasticity and behavior.

MANDEL Jean-Louis (Paris, France)
Fragile X syndrome from cloning of the Fmr1 gene to treatment

MANZONI Olivier (Bordeaux, France)
Synaptic plasticity and Mental retardation

MONYER Hannah (Heidelberg, Germany)
Postnatal neurogenesis of GABAergic interneurones

MULLER Dominique (Geneva, Switzerland)
Dendritic spine formation and stabilization.

NELSON David (Houston, USA)
Modeling Fragile X syndrome and FXTAS in mice

OERTNER Thomas (Basel, Switzerland)
Functional diversity of spine synapses 

OOSTRA Ben  (Rotterdam, The Netherlands)
Rescue of Fragile X behavioral phenotype and neuronal protrusion morphology by mGluR5 antagonists

PICKETTS David (Ottawa – Canada)
Biological consequences of depleting the epigenetic regulator ATRX

ROPERS Hans-Hilger (Berlin – Germany)
Mental retardation: large-scale genetic dissection by next generation sequencing'?

SCHENCK Annette (Nijmegen - The Netherlands)
 Modelling Mental Retardation in Drosophila - Targeted and systematic approaches

TIEDGE Henri (New York, USA)
Regulatory RNAs in neurons

TONIOLO Daniela(Milan, Italy)
Cognitive impairment in Gdi1-deficient mice

 

Deadline for application: June 20, 2010

 

Registration fee (including board and lodging)

360 € for PhD students
570 € for other participants

 

Application for registration

The total number of participants is limited to about 115 and all participants are expected to attend for the whole duration of the conference. Selection is made on the basis of the affinity of potential participants with the topics of the conference. Scientists and PhD Students interested in the meeting should send:

  • their curriculum vitae
  • the list of their main publications for the 3 last years
  • the abstract of their presentation

 

to the Chairperson of the conference before the deadline. After it, the chairman will select the participants. Except in some particular cases approved by the Chairperson, it is recommended that all selected participants present their work during the conference, either in poster form or by a brief in- session talk. The organizers choose the form in which the presentations are made. No payment will be sent with application. Information on how and when to pay will be mailed in due time to those selected.