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23. Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation. Shamburek RD; Pentchev PG; Zech LA; Blanchette-Mackie J; Carstea ED; VandenBroek JM; Cooper PS; Neufeld EB; Phair RD; Brewer HB; Brady RO; Schwartz CC J Lipid Res; 1997 Dec; 38(12):2422-35. PubMed ID: 9458266 [TBL] [Abstract][Full Text] [Related]
24. [Changes in the biosynthesis, esterification and efflux of cholesterol in fibroblasts in culture from patients with Niemann-Pick disease type C]. Mazière JC; Mazière C; Mora L; Lageron A; Polonovski C; Polonovski J C R Seances Soc Biol Fil; 1986; 180(6):669-76. PubMed ID: 2952228 [TBL] [Abstract][Full Text] [Related]
25. A defect in cholesterol esterification in Niemann-Pick disease (type C) patients. Pentchev PG; Comly ME; Kruth HS; Vanier MT; Wenger DA; Patel S; Brady RO Proc Natl Acad Sci U S A; 1985 Dec; 82(23):8247-51. PubMed ID: 3865225 [TBL] [Abstract][Full Text] [Related]
26. Cultured fibroblasts from patients with Niemann-Pick disease type C and type D exhibit distinct defects in cholesterol esterification. Sidhu HS; Rastogi SA; Byers DM; Cook HW; Palmer FB; Spence MW Biochim Biophys Acta; 1992 Feb; 1124(1):29-35. PubMed ID: 1543723 [TBL] [Abstract][Full Text] [Related]
27. Progesterone metabolism in human fibroblasts is independent of P-glycoprotein levels and Niemann-Pick type C disease. Zhang J; Ming LJ; Sjövall J; Cook HW; Ridgway ND; Byers DM J Steroid Biochem Mol Biol; 1999; 70(4-6):123-31. PubMed ID: 10622400 [TBL] [Abstract][Full Text] [Related]
28. Niemann-Pick disease: coupling and uncoupling of inhibited sphingomyelinase activity and exogenous cholesterol esterification in fibroblasts by ionophore treatment. Sakuragawa N; Mito T; Kawada A Biochim Biophys Acta; 1994 Jul; 1213(2):193-8. PubMed ID: 8025130 [TBL] [Abstract][Full Text] [Related]
29. Metabolic abnormalities in feline Niemann-Pick type C heterozygotes. Brown DE; Thrall MA; Walkley SU; Wurzelmann S; Wenger DA; Allison RW; Just CA J Inherit Metab Dis; 1996; 19(3):319-30. PubMed ID: 8803775 [TBL] [Abstract][Full Text] [Related]
30. Niemann-Pick type-C disease: deficient intracellular transport of exogenously derived cholesterol. Roff CF; Goldin E; Comly ME; Blanchette-Mackie J; Cooney A; Brady RO; Pentchev PG Am J Med Genet; 1992 Feb; 42(4):593-8. PubMed ID: 1609841 [TBL] [Abstract][Full Text] [Related]
31. Altered sensitivities to potential inhibitors of cholesterol biosynthesis in Niemann-Pick type C fibroblasts. Ohno K; Nanba E; Nakano T; Inui K; Okada S; Takeshita K Cell Struct Funct; 1993 Aug; 18(4):231-40. PubMed ID: 8293500 [TBL] [Abstract][Full Text] [Related]
32. Macrophage uptake of oxidized LDL inhibits lysosomal sphingomyelinase, thus causing the accumulation of unesterified cholesterol-sphingomyelin-rich particles in the lysosomes. A possible role for 7-Ketocholesterol. Maor I; Mandel H; Aviram M Arterioscler Thromb Vasc Biol; 1995 Sep; 15(9):1378-87. PubMed ID: 7670952 [TBL] [Abstract][Full Text] [Related]
33. Effects of drugs on cholesterol esterification in normal and Niemann-Pick type C fibroblasts: AY-9944, other cationic amphiphilic drugs and DMSO. Yoshikawa H Brain Dev; 1991; 13(2):115-20. PubMed ID: 1716424 [TBL] [Abstract][Full Text] [Related]
34. Genetic heterogeneity in Niemann-Pick C disease: a study using somatic cell hybridization and linkage analysis. Vanier MT; Duthel S; Rodriguez-Lafrasse C; Pentchev P; Carstea ED Am J Hum Genet; 1996 Jan; 58(1):118-25. PubMed ID: 8554047 [TBL] [Abstract][Full Text] [Related]
35. Cholesterol esterification and Niemann-Pick disease: an approach to identifying the defect in fibroblasts. Bowler LM; Shankaran R; Das I; Callahan JW J Neurosci Res; 1990 Dec; 27(4):505-11. PubMed ID: 2079713 [TBL] [Abstract][Full Text] [Related]
36. Filipin test for diagnosis of Niemann-Pick disease type C. Ledvinová J; Elleder M Sb Lek; 1993; 94(2):137-43. PubMed ID: 7992006 [TBL] [Abstract][Full Text] [Related]
37. A second complementation class of cholesterol transport mutants with a variant Niemann-Pick type C phenotype. Dahl NK; Daunais MA; Liscum L J Lipid Res; 1994 Oct; 35(10):1839-49. PubMed ID: 7852861 [TBL] [Abstract][Full Text] [Related]
38. Cholesterol accumulation in tissues of the Niemann-pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ. Xie C; Turley SD; Dietschy JM Proc Natl Acad Sci U S A; 1999 Oct; 96(21):11992-7. PubMed ID: 10518564 [TBL] [Abstract][Full Text] [Related]