Journal
JOURNAL OF INHERITED METABOLIC DISEASE
Volume 23, Issue 1, Pages 54-62Publisher
KLUWER ACADEMIC PUBL
DOI: 10.1023/A:1005650930330
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Niemann-Pick type C (NPC) is a neurodegenerative disorder with somatically altered cholesterol metabolism. The NPC1 gene has recently been cloned and shown to have sequences shared with known sterol-sensing proteins. We have used a mouse model with a disrupted Npc1 gene to study two cholesterol-lowering drugs (nifedipine and probucol) and the effects of introducing a null mutation in the low-density lipoprotein receptor (LDLR). Although these treatments significantly ameliorated liver cholesterol storage, little effect on the onset of neurological symptoms was found.
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