cellular
• late endosome/lysosomes are increased in size in the kidneys
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• mice show an accumulation of precursor and mature cathepsin D in the kidney, indicating trafficking defects and mis-sorting of lysosomal enzymes
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• mice show an increase in IGF2R (CI-M6PR), indicating less efficient cycling of CI-M6PR at the trans-Golgi network
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renal/urinary system
• late endosome/lysosomes are increased in size in the kidneys
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