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Biochemical features of human cystinosin. (A) Putative topology. The sequence in the C terminus corresponds to the lysosomal sorting motif. PQ-loop motifs are highlighted in blue. (B) Multiple sequence alignment of cystinosin in the PQ-loop regions. The PQ-loop consensus sequence is indicated in blue. Protonatable residues included in the neutralization scan are labeled with red stars.
