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Page 1
Cellular pregnenolone esterification by acyl-CoA:cholesterol acyltransferase.
Rogers MA, Liu J, Kushnir MM, Bryleva E, Rockwood AL, Meikle AW, Shapiro D, Vaisman BL, Remaley AT, Chang CCY, Chang TY. Rogers MA, et al. J Biol Chem. 2012 May 18;287(21):17483-17492. doi: 10.1074/jbc.M111.331306. Epub 2012 Apr 2. J Biol Chem. 2012. PMID: 22474282 Free PMC article.
Partial blockage of sterol biosynthesis with a squalene synthase inhibitor in early postnatal Niemann-Pick type C npcnih null mice brains reduces neuronal cholesterol accumulation, abrogates astrogliosis, but may inhibit myelin maturation.
Reid PC, Lin S, Vanier MT, Ohno-Iwashita Y, Harwood HJ Jr, Hickey WF, Chang CC, Chang TY. Reid PC, et al. J Neurosci Methods. 2008 Feb 15;168(1):15-25. doi: 10.1016/j.jneumeth.2007.09.002. Epub 2007 Sep 12. J Neurosci Methods. 2008. PMID: 17949821 Free PMC article.
A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.
Maue RA, Burgess RW, Wang B, Wooley CM, Seburn KL, Vanier MT, Rogers MA, Chang CC, Chang TY, Harris BT, Graber DJ, Penatti CA, Porter DM, Szwergold BS, Henderson LP, Totenhagen JW, Trouard TP, Borbon IA, Erickson RP. Maue RA, et al. Hum Mol Genet. 2012 Feb 15;21(4):730-50. doi: 10.1093/hmg/ddr505. Epub 2011 Nov 2. Hum Mol Genet. 2012. PMID: 22048958 Free PMC article.