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Publication : Glrb<sup>spa-Alb</sup> - spastic-Albany

First Author  Frost White W Year  1988
Journal  Mouse News Lett Volume  80
Pages  162-3 Mgi Jnum  J:64454
Mgi Id  MGI:1889266 Citation  Frost White W, et al. (1988) Glrbspa-Alb - spastic-Albany. Mouse News Lett 80:162-3
abstractText  Full text of MNL contribution: 4. Spastic-Albany (spaAlb): A new Spastic Allele. A new autosomal recessive mutation that produces a neurological disorder, characterized by an early defect in righting reflex, stiffened gait, progression to severe spasticity and rigidity with tremors, and death before weaning, appeared spontaneously in the C57BL/6Fla-H-2k Tla strain. These behavioral abnormalities are very similar to those seen in spastic mice, only more severe. Test matings show that this mutation is allelic to spastic (spa). Therefore, it has been named spaA1b (J. Neurogenetics 4: 253-258, 1987). The behavioral abnormalities are improved by injections of aminooxyacetic acid (AOAA), but this does not improve survival. Levels of 3H-strychnine binding in the brainstem and spinal cord of mutants are less than 10% of control values. The greater reduction in strychnine binding correlates with the greater severity of the behavioral abnormalities, when compared with spastic mice. Whether this results from differences between the mutations per se or the genetic backgrounds on which the two mutations are carried is not known at this time. Autoradiographic examination of the distribution of 3H-strychnine binding sites in both mutants confirm a greatly reduced level of binding compared to control in all areas of the spinal cord, brainstem and midbrain. (W. Frost White, Laura J. Regan and Anna W. Roe, with Anne Messer, New York State Department of Health, Albany)
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