<prompt> The protein defective in cystinosis is responsible forAbsorption of cysteine from intestineAbsorption of cysteine from renal tubulesEfflux cysteine from endoplasmic reticulumEfflux of cysteine from lysosome  <response>: The protein defective in cystinosis is Cystinosin CTNS lysosomal efflux. Clinical features:- Renal Fanconis syndrome Renal failure Rickets Photophobia Hypotonia Do not confuse cystinuria and cystinosis. Cystinosis is a lysosomal storage disease due defective transpo of cystine out of lysosomes. 