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2006 Awarded Research Projects

[Basic Research]   [Myelin Repair]    [New Gene Identification]    [Pathophysiology]   
[Therapeutic Approch]     [Others]    

Topic : Basic research
• Study of the role of cdk2 in the oligodendroglial development and identification of new genes essential for oligodendrocyte progenitors proliferation and differenciation
193 000 €
• Biochemical and Proteomic Identification of Lgi4 Binding Proteins
63 457 €
• Molecular characterization, in a model system, of the genetic relationship between pABC1 (ALDP ortholog) and PEX2
22 000 €
• Role of NADPH oxydases in the development and neurotoxic activation of microglia
14 500 €
• Role of ephrinB signaling in axon-oligodendrocyte engagement and initiation of myelination
45 000 €
• Effects of plasmalogens deficiency and very-long-chain fatty acid accumulation in myelin: a structural and biochemical study
53 000 €
Topic Myelin Repair
• Therapeutic approach of central nervous system remyelination: development of a pre-clinical model of demyelination
80 850 €
• Adult stem cells for myelin repair
30 000 €
• Analyze myelinating or remyelinating potentiality of adult bone marrow and umbilical cord stem cells in different models of leucodystrophy
102 000 €
Topic : New Gene Identification
• Identification of the gene causing leucodystrophy with ataxia, deafness and cardiomyopathy in a consanguinous italian family
64 400 €
Topic Pathophysiology
• Pathophysiological role of MLC1, a protein involved in megalencephalic leukoencephalopathy with subcortical cysts
49 500 €
• MLC disease: elucidating the role of MLC1 protein in brain physiology and physiopathogenesis; research for MLC genes
50 000 €
• PLP/DM20 alternative splicing: studies on regulatory sequences, splicing factors and mutations affecting splicing in PMD patients
41 000 €
• Role of phytanic acid in nervous system pathogenesis using mouse models for Refsum disease
95 000 €
• 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
40 000 €
• Molecular mechanisms in the pathogenesis of metachromatic leukodystrophy
87 000 €
• Elongation of very long-chain fatty acids in X-linked adrenoleukodystrophy:
an option for a therapy?
125 477 €
• Influence of methionine metabolism on phenotypic heterogeneity in X-linked adrenoleukodystrophy: An option for therapy?
10 000 €
• Study of the pathophysiological mechanisms involved in a genetic disease of the astrocyte, the Alexander disease
90 000 €
• Physiopathogenesis of X-linked adrenoleukodystrophy in the mouse. ABCD2 and ABCD4 peroxisomal transporters as therapeutic targets
80 000 €
• Analysis of degenerative mechanisms induced by very long chain fatty acids (VLCFA) in neurons and glial cells
57 500 €
• A combined magnetic resonance imaging and histological approach to understanding and treating white matter disease of prematurity
96 197 €
• Functional analysis of the ALDRP protein
25 000 €
• Proteomics for myelin (P4M) mass spectrometry platform dedicated to biomarker discovery and quantitative studies for leukodystrophies
285 295 €
• In vivo and in vitro functional analysis of the new transcripts encoded by the human PLP1 gene
50 000 €
• Invariant NKT cells in the pathophysiology of adrenoleukodystrophy
62 500 €
Topic Therapeutic Approach
• EIF2B related diseases: physiopathological and therapeutic approaches from yeast to human
272 000 €
• NMR follow-up of PLP mutant mice: application to the therapeutic evaluation of neuro-repairing molecules
60 000 €
• Correcting PLP Driven Myelin Deficiencies: Cellular therapeutic approaches to restore a normal phenotype
177 000 €
• AAV-based gene therapy for Metachromatic Leukodystrophy: preclinical steps towards a clinical application
161 300 €
• Remyelination induction by thyroid hormone based therapy and assessment by diffusion tensor magnetic resonance imaging (DT-MRI)
60 000 €
• Diffusion Tensor Imaging and Tractography of White Matter Maturation in Preterm Infants exposed to NIDCAP
67 000 €
• Homing in on novel AAV–based vectors for a leukodystrophy gene therapy
57 000 €
• Enzyme replacement therapy in a mouse model for metachromatic leukodystrophy: evaluation and optimization of enzymatic parameters
37 000 €
• Evaluation of cellular therapy in leucodystrophies with peripheral nervous system demyelination
40 000 €
• Upregulation of ABCD2/ALDR and ABCD3/PMP70 by low-fat diet, statins and fibrates as a potential therapy for X-ALD
93 925 €
• Neuroprotection in perinatal brain injury. Multi-modal approach using advanced magnetic resonance imaging, histopathological and somatosensory functional analysis
53 733 €
• Mitochondrial Impairment in Pelizaeus-Merzbacher Disease: Consequences for Therapeutic Strategies
181 800 €
• Evaluation of mesenchymal stem cells and macrophage progenitors to accelerate microglia replacement and disease arrest after hematopoietic cell transplantation in ALD
125 000 €
Others
• Set-up of a new Inserm research laboratory whose one of its principal topics is leukodystrophies therapy
115 534 €
• Therapeutic approach of central nervous system remyelination: development of a pre-clinical model of demyelination
60 000 €
• Identification and characterization of specific biomarkers in MLD and ALD using peptidomics (2nd semester 2006)
126 000 €
 
 

Total 3 599 968 €