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2006 Awarded Research Projects
Basic Research
Study of the role of cdk2 in the oligodendroglial development and identification of new genes essential for oligodendrocyte progenitors proliferation and differenciation
Dr Shibeshih Belachew - CNCM - Liège - Belgique
Pr Vittorio Gallo - Center For Neuroscience - Washington - USA
Pr Anne Baron-Van Evercooren - INSERM U546 - Université Pierre et Marie Curie - Paris 6 - France
Dr Philipp Kaldis - Center for Cancer research - Frederick - USA
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Biochemical and Proteomic Identification of Lgi4 Binding Proteins
Dr. John BERMINGHAM
Mc Laughlin Research Institute - Great Falls - USA
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Molecular characterization, in a model system, of the genetic relationship between pABC1 (ALDP ortholog) and PEX2
Dr. Véronique BERTEAUX LECELLIER - Institut de Génétique et Microbiologie - Orsay -France
Léonardo PERAZA-REYES - Institut de Génétique et Microbiologie - Orsay - France
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Role of NADPH oxydases in the development
and neurotoxic activation of microglia
Dr. Michel MALLAT - INSERM U 711 - Paris - France
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Role of ephrinB signaling in axon-oligodendrocyte engagement
and initiation of myelination
Dr. Jean-Léon THOMAS - INSERM U 711 - Paris - France
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Effects of plasmalogens deficiency and very-long-chain fatty acid
accumulation in myelin: a structural and biochemical study
Pr. Ronald WANDERS - AMC - Amsterdam - Pays Bas
Dr. Daniel KIRSCHNER - Boston College - USA
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Myelin Repair
Therapeutic approach of central nervous system remyelination:
development of a pre-clinical model of demyelination
Dr. Anne BARON VAN EVERCOOREN - INSERM U546 -
Université Pierre et Marie Curie - Paris 6 - France
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Adult stem cells for myelin repair
Dr. Pascale DURBEC - IBDML - Marseille - France
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Analyze myelinating or remyelinating potentiality of adult bone marrow and umbilical cord stem cells in different models of leucodystrophy
Dr. Isabelle DUSART - CNRS UMR 7102 - Paris - France
Pr. Salvador MARTINEZ - Institut des Neurosciences - Alicante - Espagne
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New Gene Identification
Identification of the gene causing leucodystrophy with ataxia, deafness and cardiomyopathy in a consanguinous italian family
Pr. Enrico BERTINI - Hôpital Bambino Gesus - Rome - Italie
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont-Ferrand - France
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Pathophysiology
Pathophysiological role of MLC1, a protein involved in megalencephalic leukoencephalopathy with subcortical cysts
Dr. Elena AMBROSINI - Instituto Superiore di Sanita - Rome - Italie
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MLC disease: elucidating the role of MLC1 protein in brain physiology and physiopathogenesis; research for MLC genes
Pr. Enrico BERTINI - Hôpital Bambino Gesus - Rome - Italie
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont-Ferrand - France
Dr. Meral OZGUC - Hacettepe University - Ankara - Turquie
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PLP/DM20 alternative splicing: studies on regulatory sequences,
splicing factors and mutations affecting splicing in PMD patients
Dr. Franca CAMBI - Chandler Medical Center - Lexington - USA
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont Ferrand - France
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Role of phytanic acid in nervous system pathogenesis using mouse models for Refsum disease
Dr. Sacha FERDINANDUSSE - AMC - Amsterdam - Pays Bas
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EAE induction in X-linked adrenoleukodystrophy: Effect of altered CNS (oligodendroglial) susceptibility on inflammation and demyelination in disease pathogenesis and its relationship to multiple sclerosis
Pr. Jutta GARTNER - Université Georg August - Göttingen - Allemagne
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Molecular mechanisms in the pathogenesis of metachromatic leukodystrophy
Pr. Volkmar GIESELMANN - Institut fur Physiologische Chemie - Bonn - Allemagne
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Elongation of very long-chain fatty acids in X-linked adrenoleukodystrophy:
an option for a therapy?
Dr. Stephan KEMP - AMC - Amsterdam - Pays Bas
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Influence of methionine metabolism on phenotypic heterogeneity
in X-linked adrenoleukodystrophy: An option for therapy?
Dr. Michael LINNEBANK - University Hospital Bonn - Allemagne
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Study of the pathophysiological mechanisms involved in a genetic disease of the astrocyte, the Alexander disease
Dr. Danielle PHAM-DINH - INSERM U546 - Université Pierre et Marie Curie - Paris 6 - France
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Physiopathogenesis of X-linked adrenoleukodystrophy in the mouse. ABCD2 and ABCD4 peroxisomal transporters as therapeutic targets
Dr. Aurora PUJOL - CGMM - L'Hospitalet de Llobregat - Espagne
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Analysis of degenerative mechanisms induced by very long chain fatty acids (VLCFA) in neurons and glial cells
Pr. Georg REISER - Institut fur Neurobiochemie - Magdeburg - Allemagne
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A combined magnetic resonance imaging and histological approach to understanding and treating white matter disease of prematurity
Dr. Mary Ann RUTHERFORD - Imperial College London - UK
Dr. Pierre GRESSENS - INSERM U 676 - Paris - France
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Functional analysis of the ALDRP protein
Dr. Stéphane SAVARY - LBMC - Dijon - France
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Proteomics for myelin (P4M) mass spectrometry platform dedicated to biomarker discovery and quantitative studies for leukodystrophies
Dr. Reto STOCKLIN - Atheris - Bernex - Suisse
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In vivo and in vitro functional analysis of the new transcripts encoded by the human PLP1 gene
Dr. Catherine VAURS BARRIERE - INSERM U 384 - Clermont Ferrand
- France
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Invariant NKT cells in the pathophysiology of adrenoleukodystrophy
Dr. Agnès LEHUEN - INSERM U561 - Paris - France
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Therapeutic Approach
EIF2B related diseases: physiopathological and therapeutic approaches from yeast to human
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont Ferrand - France
Dr. Reto STOCKLIN - ATHERIS - Suisse
Dr. Graham PAVITT - Faculty of Manchester - UK
Dr. Orna ELROY STEIN - University of Tel Aviv - Israel
Dr. Danielle PHAM DINH - INSERM U546 - Université Pierre et Marie Curie - Paris 6 - France
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NMR follow-up of PLP mutant mice: application to the therapeutic evaluation
of neuro-repairing molecules
Dr. Jean-Marie BONNY - STIM INRA - St Genès Champanelle - France
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Correcting PLP Driven Myelin Deficiencies: Cellular therapeutic approaches
to restore a normal phenotype
Dr. Franca CAMBI - Chandler Medical Center - Lexington - USA
Dr. Pamela KNAPP - Chandler Medical Center - Lexington - USA
Dr. Said GHANDOUR - CNRS ULP UMR7004 - Strasbourg - France
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont Ferrand - France
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AAV-based gene therapy for Metachromatic Leukodystrophy:
preclinical steps towards a clinical application
Dr. Nathalie CARTIER LACAVE - INSERM U 745 - Paris - France
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Remyelination induction by thyroid hormone based therapy and assessment
by diffusion tensor magnetic resonance imaging (DT-MRI)
Dr. Said GHANDOUR - CNRS ULP UMR7004 - Strasbourg - France
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Diffusion Tensor Imaging and Tractography of White Matter Maturation
in Preterm Infants exposed to NIDCAP
Dr. Ariel GOUT - Children Hospital - Boston - USA
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Homing in on novel AAV–based vectors for a leukodystrophy gene therapy
Dr. Matthias KLUGMANN - IZN - Heidelberg - Allemagne University of Mainz - Allemagne
Dr. Eva-Maria ALBERS - IZN - Heidelberg - Allemagne University of Mainz - Allemagne
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Enzyme replacement therapy in a mouse model for metachromatic leukodystrophy: evaluation and optimization of enzymatic parameters
Dr. Ulrich MATZNER - Physiologisch Chemisches Institut - Bonn - Allemagen
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Evaluation of cellular therapy in leucodystrophies
with peripheral nervous system demyelination
Dr. Monica MENDES SOUSA - IBMC - Porto - Portugal
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Upregulation of ABCD2/ALDR and ABCD3/PMP70 by low-fat diet,
statins and fibrates as a potential therapy for X-ALD
Dr. Bwee Tien POLL THE - AMC - Amsterdam - Pays Bas
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Neuroprotection in perinatal brain injury. Multi-modal approach using
advanced magnetic resonance imaging, histopathological
and somatosensory functional analysis
Dr. Stéphane SIZONENKO - Hôpital des Enfants - Genève - Suisse
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Mitochondrial Impairment in Pelizaeus-Merzbacher Disease:
Consequences for Therapeutic Strategies
Dr. Robert SKOFF - Wayne State University - Detroit - USA
Dr. Said GHANDOUR - CNRS ULP UMR7004 - Strasbourg - France
Pr. Odile BOESPFLUG-TANGUY - INSERM U384 - Clermont-Ferrand - France
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Evaluation of mesenchymal stem cells and macrophage progenitors
to accelerate microglia replacement and disease arrest
after hematopoietic cell transplantation in ALD
Dr. Nathalie CARTIER LACAVE - INSERM U 745 - Paris - France
Dr. Sabine FRANCOIS - INSERM U 745 - Paris - France
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Others
Set-up of a new Inserm research laboratory whose one of its principal topics
is leukodystrophies therapy
Pr. Patrick Aubourg - INSERM
Faculté des Sciences Pharmaceutiques et Biologiques
Université Paris V - France
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Therapeutic approach of central nervous system remyelination:
development of a pre-clinical model of demyelination
Dr. Anne Baron-Van Evercooren - INSERM U546 - Université Pierre et Marie Curie - Paris 6 - France
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Identification and characterization of specific biomarkers in MLD and ALD
using peptidomics (2nd semester 2006)
Dr. Reto Stöcklin - Laboratoires Atheris - Bernex - Suisse
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