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Transport of human lysosomal neuraminidase to mature lysosomes requires protective protein/cathepsin A.
Human lysosomal N-acetyl-alpha-neuraminidase is deficient in two lysosomal storage disorders, sialidosis, caused by structural mutations in the neuraminidase gene, and galactosialidosis, in which a primary defect of protective protein/cathepsin A (PPCA) leads to a combined deficiency of neuraminidas...
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| Главные авторы: | , , |
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| Формат: | Artigo |
| Язык: | Inglês |
| Опубликовано: |
European Molecular Biology Organization
1998
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| Предметы: | |
| Online-ссылка: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1170506/ https://ncbi.nlm.nih.gov/pubmed/9501080 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/emboj/17.6.1588 |
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