title: Type C Niemann-Pick disease fibroblasts and their transformed cell lines are hypersensitive to HMG-CoA reductase inhibitors.
abstract: The deficiency of exogenous cholesterol transport within fibroblasts of Niemann-Pick disease type C (NPC) has been addressed. In this report we confirmed that the endogenous synthesis of cholesterol within cells was markedly increased in NPC fibroblasts and those transformed by origin-defective simian virus 40. The transformed fibroblasts and those of the primary culture were hypersensitive to 3-hydroxy-3-methylglutaryl-coenzyme A reductase inhibitors as a consequence of their dependence on endogenous cholesterol biosynthesis. The transformed fibroblasts should help further biochemical and genetic research in this condition.
authors.affiliation: Department of Pediatrics, Jikei University, School of Medicine, Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Yamamoto
authors.forename: T
authors.initials: T
authors.lastname: Ohashi
authors.forename: T
authors.initials: T
authors.lastname: Tokoro
authors.forename: K
authors.initials: K
authors.lastname: Maekawa
authors.forename: Y
authors.initials: Y
authors.lastname: Eto
mesh_terms: D002461:Cell Line, Transformed; D005347:Fibroblasts / Q000187:drug effects* / Q000201:enzymology; D006801:Humans; D006903:Hydroxymethylglutaryl CoA Reductases / Q000378:metabolism; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D009542:Niemann-Pick Diseases / Q000201:enzymology*
chemical_list: D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D006903:Hydroxymethylglutaryl CoA Reductases