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Mucolipidosis III GNPTG missense mutations cause misfolding of the γ subunit of GlcNAc-1-phosphotransferase

The lysosomal storage disorder mucolipidosis III γ is caused by defects in the γ subunit of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase, the enzyme that tags lysosomal enzymes with the mannose 6-phosphate lysosomal targeting signal. In patients with this disorder, most of th...

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Detalhes bibliográficos
Publicado no:Hum Mutat
Main Authors: van Meel, Eline, Kornfeld, Stuart
Formato: Artigo
Idioma:Inglês
Publicado em: 2016
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4907843/
https://ncbi.nlm.nih.gov/pubmed/27038293
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/humu.22993
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