Tom Schirris, Dept. of Pharmacology and Toxicology, theme Mitochondrial diseases in collaboration with RIMLS researchers Frans Russel, Jan Smeitink, and Ria de Haas, has been awarded a 250.000 euros grant from the Prinses Beatrix Spierfonds to explore pharmacological stimulation of the cholesterol efflux in mitochondrial CI-deficiencies, in a project entitled ‘Improving complex I-deficiency by pharmacological cholesterol efflux stimulation’.
Mitochondrial complex I deficiencies are disorders of varying etiology and age of onset, and include a variety of more or less severe often multisystemic phenotypes, but typically include muscle and neurological defects.
The awarded grant money will be used to evaluate potential pharmacological interventions that stimulate the cellular cholesterol efflux, as this mechanism was recently observed to have beneficial effects on the cellular redox status of complex I-deficient patient-derived cells.
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