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Programmes de recherche financés par la Fondation ELA en 2005

[Basic research]   [Myelin Repair]    [Gene Identification]    [Physiopathology]   
[Treatment Approaches]    [Investment in Equipment]  

Basic research
• Study of the role of cdk2 in oligodendroglial development and identification of new genes essential to the proliferation and the differentiation of oligodendrocyte progenitors
183 000 €
• Diseases linked to EIF2B: Physiopathology and treatment approaches, conservation between yeasts and humans
302 468 €
• Role of Slit proteins in the recruitment and remyelinization potential of adult neural stem cells
25 000 €
• Identification of transcriptional factors controlling the specification of oligodendrocyte progenitors
20 000 €
Myelin Repair
• Promoting the formation of primate oligodendrocyte progenitors to allow the repair of CNS myelin
50 000 €
• Study of the myelinizing potential of cells from different telencephalic regions in human and non-human primates: Towards a cellular therapy of diseases affecting myelin in the central nervous system
25 000 €
Gene Identification
• Identification of a gene for leucodystrophy of unknown etiology on chromosome 11q14.3
34 050 €
• Elucidation of the role of the MLC1 protein in brain physiology and its implication in MLC pathogenesis; looking for a second gene linked to MLC1
75 000 €
• Looking for a gene implicated in a new form of leucodystrophy causing liver damage, deafness, and cardiomyopathy
64 400 €
• Molecular flaws implicated in Pelizaeus-Merzbacher-like disease
75 000 €
Physiopathology
• Analysis of myelin membrane transmission pathways in diseases linked to PLP
54 000 €
• Molecular characterization of the genetic relationship observed between pABC1 (an ortholog of ALDP) and PEX2 in a model system
25 000 €
• Role of phytanic acid in nervous system pathogenesis and mouse models of Refsum disease
72 000 €
• Rhizomelic chondrodysplasia punctata and the role of plasmalogens in its pathogenesis, including the abnormal formation of myelin
82 046 €
• Physiopathogenesis of X-linked ALD in murine models, strategies for treatment
101 000 €
• Analysis of degenerative mechanisms induced by VLCFAs in neurons and glial cells
57 500 €
• Function and regulation of the ABCD2 (ALDR) gene
30 000 €
• Identification of new exons in the human PLP1 gene: Characterization and functional study of new transcripts, study of their implication in human pathology
55 700 €
• Periventricular leucomalacy and cerebral abnormalities in premature babies: Using tractography to describe the mechanisms involved
67 000 €
• Study of the physiopathological mechanisms involved in Alexander disease, a genetic astrocyte disorder
83 000 €
• Identification and characterization of specific biomarkers for MLD and ALD using peptidomic studies
273 500 €
Treatment Approaches
• NMR follow-up of PLP-mutated mice: Use in the therapeutic evaluation of neuroreparative substances
60 000 €
• Correction of myelin deficiencies due to PLP: Cellular therapy approaches for restoring normal phenotype
177 000 €
• Gene therapy for AMN and cerebral ALD using hematopoietic stem cells corrected ex vivo with a lentiviral vector
38 074 €
• Gene therapy using AAV for the treatment of MLD: Preclinical steps towards clinical use
90 300 €
• Detection of dysmyelinization and repair of myelin using diffusion tensor MRI in the brains of transgenic mice
68 000 €
• Enzyme replacement therapy in a mouse model for MLD: Evaluation and optimization of enzymatic parameters
37 000 €
• Evaluation of cellular therapy in leucodystrophies with demyelinization of the peripheral nervous system
40 000 €
• Up-regulation of ABCD2/ALDR and ABCD3/PMP70 as a result of a low-fat diet, statins and fibrates as potential means of treating X-ALD
40 000 €
• Gene therapy for MLD: Study of MLD’s natural history and direction of preclinical studies in order to submit a protocol to AFSSAPS [French agency ensuring the safety of health products]
10 920 €
• Administration of ZK230211 (anti-progesterone) to reduce overexpression of PLP in a mouse model for Pelizaeus-Merzbacher disease
30 000 €
• Pharmacological gene therapy for X-ALD
100 000 €
• Elongation of very long-chain fatty acids in X-ALD: Are there therapeutic possibilities?
127 771 €
Investment in Equipment
• Purchase of 2 mass spectrometers
150 000 €
• Purchase of a 128-cage ventilated mouse rack
30 636 €
• CellGro culture media
22 126 €
 

Total 2 776 491 €