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- W2022138847 abstract "Summary Niemann‐Pick disease type C (NPC) is a neurovisceral storage disorder with an unknown primary deficiency. Somatic cell hybridization experiments using human cultured fibroblasts have shown that two complementation groups (NPC‐ α and NPC‐ β ) are associated with the biochemical and clinical phenotypes comprising NPC. We identified the rarer complementation group NPC‐ β originally using the technique of filipin staining as a marker for complementation. In this study we show that the esterification of cholesterol derived from the LDL pathway can be used as an isotopic assay. However, multinuclear hybrids exhibit a delayed induction in this pathway. Furthermore, we discovered that, in the presence of an LDL source, co‐cultivation of fibroblasts belonging to NPC‐ α and NPC‐ β stimulated cholesterol esterification." @default.
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- W2022138847 date "1996-02-20" @default.
- W2022138847 modified "2023-09-25" @default.
- W2022138847 title "Co‐cultivation of Niemann‐Pick disease type C fibroblasts belonging to complementation groups <i>α</i> and <i>β</i> stimulates LDL‐derived cholesterol esterification" @default.
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- W2022138847 doi "https://doi.org/10.1007/bf01799171" @default.
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