Abstract：
:Niemann-Pick disease, type C (NP-C), often associated with Niemann-Pick disease, type C1 (NPC1) mutations, is a cholesterol-storage disorder characterized by cellular lipid accumulation, neurodegeneration, and reduced steroid production. To study NPC1 function in vivo, we cloned zebrafish npc1 and analyzed its gene expression and activity by reducing Npc1 protein with morpholino (MO)-oligonucleotides. Filipin staining in npc1-morphant cells was punctate, suggesting abnormal accumulation of cholesterol. Developmentally, reducing Npc1 did not disrupt early cell fate or survival; however, early morphogenetic movements were delayed, and the actin cytoskeleton network was abnormal. MO-induced defects were rescued with ectopic expression of mouse NPC1, demonstrating functional gene conservation, and by treatments with steroids pregnenolone or dexamethasone, suggesting that reduced steroidogenesis contributed to abnormal cell movements. Cell death was found in anterior tissues of npc1 morphants at later stages, consistent with findings in mammals. Collectively, these studies show that npc1 is required early for proper cell movement and cholesterol localization and later for cell survival.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Schwend T,Loucks EJ,Snyder D,Ahlgren SCdoi
10.1194/jlr.M012377subject
Has Abstractpub_date
2011-07-01 00:00:00pages
1328-44issue
7eissn
0022-2275issn
1539-7262pii
jlr.M012377journal_volume
52pub_type
杂志文章abstract：:Several recent reports have examined whether there is a correlation between the presence of some minor alleles of the highly polymorphic apolipoprotein B gene and atherosclerosis and premature heart disease. The present study extends this investigation. A high-resolution method was used to study the allele frequencies...
journal_title：Journal of lipid research
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journal_title：Journal of lipid research
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journal_title：Journal of lipid research
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更新日期：2002-07-01 00:00:00
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更新日期：2010-07-01 00:00:00
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