| First Author | Ohno K | Year | 1992 |
| Journal | Cell Struct Funct | Volume | 17 |
| Issue | 4 | Pages | 229-35 |
| PubMed ID | 1394466 | Mgi Jnum | J:3691 |
| Mgi Id | MGI:52200 | Doi | 10.1247/csf.17.229 |
| Citation | Ohno K, et al. (1992) A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease. Cell Struct Funct 17(4):229-35 |
| abstractText | 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 homozygous embryo showed extensive accumulation of intracellular cholesterol, attenuated esterification of exogenously added cholesterol and increased de novo cholesterol synthesis, when compared to SPMH-3T3 cells derived from a heterozygous embryo. The phenotypic abnormalities were very similar to those observed in fibroblasts 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-3T3 cell line is useful for biochemical and genetic studies on the regulation of intracellular cholesterol metabolism. |
