| First Author | Neumann P | Year | 1988 |
| Journal | Mouse News Lett | Volume | 81 |
| Pages | 59-60 | Mgi Jnum | J:14139 |
| Mgi Id | MGI:62314 | Citation | Neumann P, et al. (1988) Unsteady: a new neurologic mutant. Mouse News Lett 81:59-60 |
| abstractText | Full text of MNL contribution: Research News: 2. Unsteady: A new neurologic mutant. A new recessive mutation, unsteady (uns), appeared in descendents of a cross between CD-1 outbred and C57BL/6J inbred strains. Mutant mice are smaller than littermates. Ataxic gait is evident by three weeks of age. The gait disorder is progressive; homozygotes become wobbly at rest and in motion. By three months of age, mutants have a hunched posture and their hind limbs are atrophic. Homozygotes have survived up to six months of age, but have not bred. Careful observation of mutants aged three months or more, reveals frequent short episodes of motor arrest that appear to be "staring" episodes, similar to those seen in tottering (tg) and lethargic (lh). Electrocorticographic (ECoG) recordings of unsteady mutant mice, aged two months or more, reveal single high-amplitude spike-and-wave complexes at approximately thirty-second intervals. This is in contrast to the bursts of spike-and-wave activity seen in tg and lh mutants. The neuropathologic findings are unique, suggesting that this mutation is not allelic to any that have been previously described. Axonal degeneration is prominent in the deep cerebellar and lateral vestibular nuclei. Although these fibers are identifiable as Purkinje cell projections, the Purkinje cell bodies appear unremarkable by light and electron microscopy. Selective silver impregnation (Fink-Heimer method)... (Paul Neumann, Stephen DiFazio, Craig Applegate and Richard Sidman, with Eric Sinn and Phil Leder, Department of Genetics, Harvard Medical School). |
