Fig. 4: Role of PQ motifs in cystinosin.
From: Structural basis for proton coupled cystine transport by cystinosin
a Effect of mutating the PQ motifs in both human and plant (A. thaliana) cystinosin. The human protein was assayed using TEVC (n = 109 independent experiments for H2O, n = 113 for WT, n = 22 for P144A and n = 31 for P283A) whereas the plant transporter was analysed in reconstituted liposomes (n = 9 independent experiments for WT and n = 3 for variants). Error bars SEM. b Representative KM of plant cystinosin variant Tyr167Phe. KM was calculated from three independent experiments, error SD. c Summary data showing the effect of mutations on the proposed luminal lock of human cystinosin expressed in Xenopus oocytes. n = 109 independent experiments for H2O, n = 113 for WT, n = 35 for D205A, n = 34 for Q315A, n = 22 for Q315N, n = 27 for K335A, error bars SEM. Source data are provided as a Source Data file.
