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"Lysosomal amino acid transporters"
Abstract :
Lysosomes recently gained renewed interest due to their role in the pathogenesis of neurodegenerative diseases and in nutrient sensing by
the master cell regulator ‘target of rapamycin’ (TOR). Yet basic aspects of these organelles remain unknown, especially at membrane level. Our recent research focused on the discovery and characterization of membrane proteins responsible for the export of lysosomal metabolites. These transport proteins offer a therapeutic route to rescue small-molecule
storage in some lysosomal diseases.
Selected publications :
Jézégou et al, Heptahelical protein PQLC2 is a lysosomal cationic amino acid exporter underlying the action of cysteamine in cystinosis therapy,
PNAS 2012.
Chapel et al, An extended proteome map of the lysosomal membrane reveals novel potential transporters, Mol Cell Proteomics 2013.
Ruivo e al, Mechanism of proton/substrate coupling in the heptahelical lysosomal transporter cystinosin, PNAS 2012.
Bruno Gasnier, Laboratoire de Dynamique Membranaire et Maladies
Neurologiques, Université Paris Descartes