| First Author | Sidman RL | Year | 1985 |
| Journal | Proc Natl Acad Sci U S A | Volume | 82 |
| Issue | 1 | Pages | 253-7 |
| PubMed ID | 3855546 | Mgi Jnum | J:7723 |
| Mgi Id | MGI:56192 | Doi | 10.1073/pnas.82.1.253 |
| Citation | Sidman RL, et al. (1985) Transmissible spongiform encephalopathy in the gray tremor mutant mouse. Proc Natl Acad Sci U S A 82(1):253-7 |
| abstractText | Gray tremor (gt) is an autosomal recessive mutation in the mouse linked to caracul (Ca) on chromosome 15. The complex mutant phenotype includes pigmentation defects, tremor, seizures, hypo- and dysmyelination in central and peripheral nervous systems, spongiform encephalopathy, and early death. The heterozygote (+/gt) is phenotypically normal but develops a mild spongiform encephalopathy from 2 months of age onward. The pigmentation and myelination disorders indicate that the gt genetic locus is active neonatally and probably earlier. This report focuses mainly on the later-expressed vacuolating disorder, which most closely mimics in tissue distribution, histopathology, and ultrastructure the spongiform encephalopathies caused by unconventional transmissible agents. This lesion was produced in genetically normal mice in a transmission experiment: of 99 neonatal mice inoculated intracerebrally with gt/gt brain homogenate, all 7 mice of three strains (BALB/cBy, C3HeB/FeJ, and C57BL/6J) allowed to survive for the unusually long interval of 682-721 days after inoculation, developed spongiform changes distributed as in the mutant phenotype. The gray tremor mutant presents a naturally occurring spongiform encephalopathy whose expression is determined by the interaction of genetic factors and a transmissible agent. |
