| First Author | Miyawaki S | Year | 1983 |
| Journal | J Hered | Volume | 74 |
| Issue | 6 | Pages | 465-8 |
| PubMed ID | 6315811 | Mgi Jnum | J:7245 |
| Mgi Id | MGI:55716 | Doi | 10.1093/oxfordjournals.jhered.a109840 |
| Citation | Miyawaki S, et al. (1983) Time course of hepatic lipids accumulation in a strain of mice with an inherited deficiency of sphingomyelinase. J Hered 74(6):465-8 |
| abstractText | Sphingomyelinosis (gene symbol, spm) is a recessive autosomal mutation in mice that causes a condition analogous to the human disease known as Niemann-Pick disease. The time course of hepatic lipids accumulation in this murine model was investigated. Hepatosplenomegaly in spm/ spm mice was noticeable as early as 4 weeks of age, and reached its maximum level at 6 weeks of age. Thereafter the weights of liver and spleen decreased in parallel with a decrease in body weight and an increase in severity of neurological symptoms. Hepatic concentrations of unesterified cholesterol and sphingomyelin were considerably elevated by 4 weeks of age, and further increased linearly to the terminal stages of the disorder. Sphingomyelinase activities in the livers of spm/ + and +/+ mice showed normal adult levels from as early as 4 weeks of age, whereas the activity in spm/ spm mice was consistently 30-40 percent of the normal level from 4 to 12 weeks of age. |
