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Lysosomal Storage Causes Cellular Dysfunction in Mucolipidosis II Skin Fibroblasts

Mucolipidosis II (ML-II) is a fatal inherited metabolic disease caused by deficiency of GlcNAc-phosphotransferase, which plays a role in generating the mannose 6-phosphate recognition marker on lysosomal enzymes. In ML-II, many lysosomal acid hydrolases are mistargeted out of cells, and lysosomes be...

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Autori principali: Otomo, Takanobu, Higaki, Katsumi, Nanba, Eiji, Ozono, Keiichi, Sakai, Norio
Natura: Artigo
Lingua:Inglês
Pubblicazione: American Society for Biochemistry and Molecular Biology 2011
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3186395/
https://ncbi.nlm.nih.gov/pubmed/21846724
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M111.267930
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