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The integrity of a cholesterol-binding pocket in Niemann–Pick C2 protein is necessary to control lysosome cholesterol levels
The neurodegenerative disease Niemann–Pick Type C2 (NPC2) results from mutations in the NPC2 (HE1) gene that cause abnormally high cholesterol accumulation in cells. We find that purified NPC2, a secreted soluble protein, binds cholesterol specifically with a much higher affinity (K(d) = 30–50 nM) t...
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| Главные авторы: | , , , |
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| Формат: | Artigo |
| Язык: | Inglês |
| Опубликовано: |
The National Academy of Sciences
2003
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| Предметы: | |
| Online-ссылка: | https://ncbi.nlm.nih.gov/pmc/articles/PMC151373/ https://ncbi.nlm.nih.gov/pubmed/12591949 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0530027100 |
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