Amino acid transporters play a key role controlling the flow of nutrients across the lysosomal
membrane and regulating metabolism in the cell. Mutations in the gene encoding the
transporter cystinosin result in cystinosis, an autosomal recessive metabolic disorder
characterised by the accumulation of cystine crystals in the lysosome. Cystinosin is a
member of the PQ-loop family of solute carrier (SLC) transporters and uses the proton
gradient to drive cystine export into the cytoplasm. However, the molecular basis for …

Amino acid transporters play a key role controlling the flow of nutrients across the lysosomal
membrane and regulating metabolism in the cell. Mutations in the gene encoding the
transporter cystinosin result in cystinosis, an autosomal recessive metabolic disorder
characterised by the accumulation of cystine crystals in the lysosome. Cystinosin is a
member of the PQ-loop family of solute carrier (SLC) transporters and uses the proton
gradient to drive cystine export into the cytoplasm. However, the molecular basis for …