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2007 Awarded Research Projects

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

Topic : Basic research
• Imaging myelination and demyelination
50 000 €
• A role for Sox17 in oligodendrocyte development and regeneration in the mouse and human brain
145 00 €
• the role of oligodendroglial exosome secretion in glia to axon signalling and axon protection
82 080 €
• Understanding glutamate and ATP signalling pathways involved in oligodendrocyte death
46 000 €
• PPAR-γ agonists as potential therapeutic agents to protect oligodendrocytes and promote myelination
29 000 €
• Molecular characterization, in a model system, of the genetic relationship between pABC1 (ALDP ortholog) and the peroxisomal RING-finger complex
41 043 €
• Analysis of VLCFA (very long chain fatty acid)-induced cell-toxic mechanisms triggering myelin degeneration in X-ALD
28 350 €
• Is PICK1 involved in the pathogenesis of excitotoxic injury to oligodendrocytes in PVL?
43 479 €
• New PLP1 gene mRNAs isoforms: translation study and expression in a “PLP1 humanized” transgenic mouse model
28 000 €
• Effects of plasmalogens deficiency and very-long-chain fatty acid accumulation in myelin: a structural and biochemical study
106 190 €
Topic: Myelin Repair
• Exploring the requirement of Cdk2 for normal white matter development and myelin repair
30 000 €
• Determining the neural population with the greatest capacity for myelin repair
30 515 €
• Enhancing remyelination in the ageing CNS
22 000 €
• The role of the adhesion molecule TAG-1 in glial cell function
30 000 €
• Myelin repair : The role of gender and androgens
102 000 €
• Imaging CNS demyelination and myelin repair in Multiple Sclerosis: a PET study with 11C-2-(4’-methyl-aminophenyl)-6-hydroxybenzothiazole (11C-PIB)
50 000 €
• Therapeutic approach of central nervous system remyelination: development of a pre-clinical model of demyelination
80 850 €
• Analyze myelinating or remyelinating potentiality of adult bone marrow and umbilical cord stem cells in different models of leukodystrophy
102 000 €
Topic: Identification of new genes
• Localization and identification of the gene responsible of leukodystrophy and oligodontia in a large family
20 650 €
Topic: Pathophysiopathology
• Identification of modifier genes in X-ALD
38 500 €
• Role of peroxisomes in the formation and maintenance of myelinated axons
34 870 €
• Biochemical and genetical analyses of the MLC1 interactome
64 775 €
• Mitochondrial dysfunction and oxidative stress underlying X-linked adrenoleukodystrophy (X-ALD) physiopathogenesis
44 098 €
• Impact of inflammatory cytokines on developing oligodendrocytes in a mouse model of white matter diseases of the premature infant
98 000 €
• The role of VLCFA in the pathogenesis of X-ALD: generation of an ELOVL1 over-expressing X-ALD mouse
44 000 €
• The role of mitochondrial respiration on myelination and axon-glia interaction
20 592 €
• Alexander disease: Pathophysiological mechanisms using mutant GFAP Knock-in models
90 000 €
• 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
28 000 €
• Role of phytanic acid in nervous system pathogenesis using mouse models for Refsum disease
31 271 €
• Molecular mechanisms in the pathogenesis of metachromatic leukodystrophy
74 000 €
• Elongation of very long-chain fatty acids in X-linked adrenoleukodystrophy: an option for a therapy?
125 309 €
• Physiopathogenesis of X-linked adrenoleukodystrophy in the mouse. ABCD2 and ABCD4 peroxisomal transporters as therapeutic targets
83 000 €
• A combined magnetic resonance imaging and histological approach to understanding and treating white matter disease of prematurity
93 200 €
• Functional analysis of the ALDRP protein
25 000 €
• Invariant NKT cells in the pathophysiology of adrenoleukodystrophy
62 100 €
• Proteomics for myelin (P4M) mass spectrometry platform dedicated to biomarker discovery and quantitative studies for leukodystrophies
285 295 €
Topic: Therapeutic Approch
• Gene therapy for X-ALD: First step for the production of the lentiviral vector
160 000 €
• Arylsulfatase A over-expression in human hematopoietic cells for MLD gene therapy: pre-clinical and clinical assessment
80 000 €
• Mitochondrial Impairment in Pelizaeus-Merzbacher Disease: Consequences for Therapeutic Strategies
181 8 00 €
• Evaluation of mesenchymal stem cells and macrophage progenitors to accelerate microglia replacement and disease arrest after hematopoietic cell transplantation in ALD
101 000 €
• Participation to a platform in Nantes producing viral vectors for clinical applications
200 000 €
• POPART’MUS: Prevention of Post Partum Relapses with Progestin and Estradiol in Multiple Sclerosis
93 000 €
• Enzyme replacement therapy in an improved metachromatic leukodystrophy mouse model
47 000 €
• Newborn Screening for X-Linked Adrenoleukodystrophy and other Peroxisomal Disorders
42 397 €
• Intracerebral administration of adeno-associated viral vector carrying the human ARSA cDNA in metachromatic leukodystrophy
163 800 €
• EIF2B related diseases: physiopathological and therapeutic approaches from yeast to human
260 326 €
• Homing in on novel AAV–based vectors for a leukodystrophy gene therapy
51 800 €
• Neuroprotection in perinatal brain injury. Multi-modal approach using advanced magnetic resonance imaging, histopathological and somatosensory functional analysis
53 733 €
• Set-up and development of gene therapy clinical trial in X-linked adrenoleukodystrophy
55 000 €
 

Total 3 848 523 €