CTNS and cystinosis: The accumulation of the autophagy substrate SQSTM1, which is normally degraded within autolysosomes, suggests an abnormal autophagy flux in cystinosin-deficient PT cells, in line with recent observations stating an impairment of autophagy flux in many lysosome storage diseases37,38 and evidence in human cells and kidney biopsies from cystinosis patients16.