These mice carry a spontaneous mutation at the Glra1 locus characterized by a fine motor tremor and muscle spasms that begin at 2 weeks of age and become progressively severe, resulting in death by 3 weeks of age.Read More +
The spasmodic oscillator mouse carries a 9 bp microdeletion plus a 2 bp microinsertion in the glycine receptor alpha1 subunit gene, resulting in the absence of functional alpha1 polypeptides from the central nervous system and lethality 3 weeks after birth. Affected animals begin to tremble violently and very rapidly at about 14 days of age. Severity of the tremor increases daily. At 19-21 days the affected animals have prolonged periods of great rigor or stiffness. Membranes isolated from oscillator homozygote spinal cords display a 90% reduction in glycine-displaceable strychnine binding. Mutations which impair GLRA1 function in humans have been shown to cause dominant familial startle disease.
When maintaining a live colony, heterozygous mice may be bred to wildtype mice from the colony or to C57BL/6J inbred mice (Stock No. 000664). Homozygotes live less than 20 days.
When using the spasmodic oscillator mouse strain in a publication, please cite the originating article(s) and include JAX stock #000536 in your Materials and Methods section.