A Cell Line Derived from Sphingomyelinosis Mouse Shows Alterations in Intracellular Cholesterol Metabolism Similar to Those in Type C Niemann-Pick Disease

Cell Structure and Function

Kenzo Takeshita²  Shigeki Miyawaki³  Eiji Nanba²  Takeshi Sakiyama¹  Teruo Kitagawa¹  Kousaku Ohno² 

[1] Department of Pediatrics, Nihon University School of Medicine;Division of Child Neurology, Institute of Neurological Sciences, Tottori University School of Medicine;Research Laboratories, Nippon Shinyaku Co. Ltd.

关键词: sphingomyelinosis;    Niemann-Pick disease;    cholesterol;    cholesterol transport;   

DOI  :  10.1247/csf.17.229

学科分类：分子生物学，细胞生物学和基因

来源: Japan Society for Cell Biology

【 摘 要 】

References(26)Cited-By(6)Cell lines derived from the sphingomyelinosis (gene symbol, spm) mouse were established from homozygous (spm/spm) and heterozygous (spm/+) embryos according to a rigid 3T3 transfer schedule. The SPM-3T3 cells derived from a homozygousembryo showed extensive accumulation of intracellular cholesterol, attenuated esterification of exogenously added cholesterol and increased de novo cholesterol synthesis, when compared to SPMH-3T3cells derived from a heterozygous embryo. The phenotypic abnormalities were very similar to those observed in fibrobJasts from patients with Niemann-Pick disease type C (NP-C), in which a defect in the intracellular transport of unesterified cholesterol is suggested. The genetic defect in SPM-3T3 cells should be closely related to that in NP-C. The SPM-3T3cell line is useful for biochemical and genetic studies on the regulation of intracellular cholesterol metabolism.

【 授权许可】

Unknown