By 2 weeks of age, mice homozygous for the scrambler 3 Jackson mutation are smaller than their littermates and ataxic with a severely unstable gait and inability to right themselves. They rarely survive beyond 3 weeks of age, although heterozygotes have a normal lifespan. Histology of homozygotes shows disorganization of the hippocampal layers and a lack of cerebellar folia. The scrambler 3 Jackson mutation causes a more severe phenotype than does the scrambler 2 Jackson mutation.
The scrambler 3 Jackson mutation arose spontaneously in the F2 offspring of a linkage cross of B6.CAST-Cdh23Ahl+/Kjn and B6.129S7-Sod1tm1Leb/DnJ and was backcrossed twice to C57BL/6J before being maintained by sibling intercrossing homozygous ovarian transplant hosts with heterozygous males. The targeted mutation has been bred out of this mutant subline.
|Allele Name||scrambler 3 Jackson|
|Gene Symbol and Name||Dab1, disabled 1|
|Strain of Origin||(B6.CAST-Cdh23+/Kjn x B6.129S7-Sod1tm1Leb/DnJ)F2|
|Molecular Note||This spontaneous mutation arose spontaneously at The Jackson Laboratory and was proven allelic with the original scrambler mutation by failed complementation.|