Lysosomal Proteome and Secretome Analysis Identifies Missorted Enzymes and Their Nondegraded Substrates in Mucolipidosis III Mouse Cells

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Di Lorenzo, Giorgia; Velho, Renata Voltolini; Winter, Dominic; Thelen, Melanie; Ahmadi, Shiva; Schweizer, Michaela; De Pace, Raffaella; Cornils, Kerstin; Yorgan, Timur Alexander; Grüb, Saskia; Hermans-Borgmeyer, Irm; Schinke, Thorsten; Müller-Loennies, Sven; Braulke, Thomas; Pohl, Sandra;

Targeting of soluble lysosomal enzymes requires mannose 6-phosphate (M6P) signals whose formation is initiated by the hexameric N-acetylglucosamine (GlcNAc)-1-phosphotransferase complex (α(2)β(2)γ(2)). Upon proteolytic cleavage by site-1 protease, the α/β-subunit precur... View more
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