valinomycin and Cystinosis

This paper describes the stimulation of exodus of cystine from lysosome-rich granular fractions by potassium. Potassium permeability into lysosomes is low, but in the presence of an ionophore or permeable anion, the movement of K+ into lysosomes caused a large stimulation of cystine exodus. Lysosomal preparations from leucocytes of cystinotic patients, which lack carrier-mediated cystine transport, also manifested stimulation of cystine egress by valinomycin and K+. This suggests that potassium-dependent cystine egress involves a carrier different from that defective in cystinosis, or occurs through a non-carrier-mediated mechanism.

Topics: Cations, Monovalent; Cell Membrane Permeability; Cystine; Cystinosis; Cytoplasmic Granules; Humans; Leukocytes; Lysosomes; Nigericin; Potassium; Valinomycin