Mice homozygous for the staggerer spontaneous mutation (Rorasg) show a staggering gait, mild tremor, hypotonia, and small size. The cerebellar cortex of homozygous mutant mice is grossly underdeveloped with a deficiency of granule cells and Purkinje cells. The remaining granule cells migrate inward from the external layer prematurely and then degenerate. Purkinje cells are much delayed in postnatal differentiation and lack the dendritic spines on which synapses with the parallel fibers from the granule cells normally occur. Staggerer mutant mice have been used as a source of an agranulate cerebellum in a number of investigations of the composition and function of granule cells. Kopmels et al. have reported a hyperproduction of IL1 biological activity and mRNA from LPS stimulated spleen cells of Rorasg/Rorasg mice on the C57BL/6J background relative to wild type siblings.
In this congenic strain the staggerer mutation is maint...
Genetic Background | Generation |
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000664 C57BL/6J |
|
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Spontaneous | Myo5a | myosin VA |
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Spontaneous | Bmp5 | bone morphogenetic protein 5 |
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Spontaneous | Rora | RAR-related orphan receptor alpha |
Mice homozygous for the staggerer spontaneous mutation (Rorasg) show a staggering gait, mild tremor, hypotonia, and small size. The cerebellar cortex of homozygous mutant mice is grossly underdeveloped with a deficiency of granule cells and Purkinje cells. The remaining granule cells migrate inward from the external layer prematurely and then degenerate. Purkinje cells are much delayed in postnatal differentiation and lack the dendritic spines on which synapses with the parallel fibers from the granule cells normally occur. Staggerer mutant mice have been used as a source of an agranulate cerebellum in a number of investigations of the composition and function of granule cells. Kopmels et al. have reported a hyperproduction of IL1 biological activity and mRNA from LPS stimulated spleen cells of Rorasg/Rorasg mice on the C57BL/6J background relative to wild type siblings.
In this congenic strain the staggerer mutation is maintained in repulsionwith both the dilute (Myo5ad) and short ear (Bmp5se) mutations.
The first Rorasg/Rorasg mouse was observed in 1955 among the F2 progeny of a (BALB/cHm x C3H/HeJ)F1 female and a male of an obese (Lepob) stock of mixed background. The mutation was maintained for several generations by an intercross-backcross (within the same or a parallel lineage) mating scheme, then was backcrossed onto C57BL/6J for four generations. A Rorasg/+ male from the fourth backcross was mated to a female C57BL/10-Myo5ad Bmp5se mouse to introduce the dilute and short-ear mutations into the stock in repulsion with Rora6 sg; this allowed the heterozygotes (Rorasg + +/+ Myo5ad Bmp5se to be identified by a lack of either recessive phenotype. Brother-sister inbreeding was continued. In 1978, which the line was at generation F55, Myo5ad Bmpse was backcrossed onto C57BL/6J seven times through a two generation backcross-intercross breeding scheme; at F58, Rorasg was backcrossed onto C57BL/6J four times also by backcross-intercross. The Rorasg + +/+ Myo5ad Bmp5se stock was then reconstructed by crossing mice of the two independent stocks and maintained by brother-sister inbreeding. In 1982, after 4 or 5 generations of sibling crosses, embryos were generated for cryopreservation from proven Rorasg + +/+ Myo5ad Bmp5se repulsion heterozygous males bred to females that were likely also repulsion heterozygotes. The recovered pups are expected to be either homozygous for Rorasg and wildtype for dilute and short ear, wildtype for Rora and homozygous for dilute or short ear, or have no phenotype and be repulsion heterozygotes Rorasg + +/+ Myo5ad Bmp5se.
Allele Name | dilute |
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Allele Type | Spontaneous |
Allele Synonym(s) | blue dilution; d; dv; Maltese dilution |
Gene Symbol and Name | Myo5a, myosin VA |
Gene Synonym(s) | |
Strain of Origin | old mutant of the mouse fancy |
Chromosome | 9 |
Molecular Note | This mutation is the result of the integration of ecotropic murine leukemia virus Emv-3 into a noncoding region of the Myo5ad gene. Reversions of Myo5ad to wild-type are caused by excision of the virus leaving exactly one long terminal repeat in place. |
Allele Name | short ear |
---|---|
Allele Type | Spontaneous |
Allele Synonym(s) | seGnJ |
Gene Symbol and Name | Bmp5, bone morphogenetic protein 5 |
Gene Synonym(s) | |
Strain of Origin | mice from Abbie Lathrop mouse farm |
Chromosome | 9 |
General Note | Phenotypic Similarity to Human Syndrome: Ear, Patella, Short Stature Syndrome (Meier-Gorlin Syndrome) in homozygous mice (J:24474) |
Molecular Note | The C to T substitution creates a stop codon at arginine codon 208 (p.R208*). The resulting truncated protein does not include the carboxy terminal signaling portion of the molecule. |
Allele Name | staggerer |
---|---|
Allele Type | Spontaneous |
Allele Synonym(s) | RORalpha-; sg |
Gene Symbol and Name | Rora, RAR-related orphan receptor alpha |
Gene Synonym(s) | |
Strain of Origin | obese stock |
Chromosome | 9 |
Molecular Note | This allele contains a 6.5kb genomic deletion of an exon encoding part of the ligand binding domain. The deletion results in an exon-skipping event that introduces a shift in the reading frame. The resulting protein is predicted to be truncated due to introduction of a premature stop codon. |
Rorasg/Rorasg mice can be identified by 10-15 days of age by their poor locomotor coordination, lurching movements and frequent falls; homozygotes as young as three days can be distinguished by behavioral testing (Heuze et al.1997). Myo5ad Bmp5se homozygotes can be identified by short ears and a slate gray coat color.
When using the B6.Cg-Rorasg + +/+ Myo5ad Bmp5se/J mouse strain in a publication, please cite the originating article(s) and include JAX stock #000285 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Heterozygous or Wild-type for Rora<sg>, Heterozygous or Wild-type for Myo5a<d>, Heterozygous or Wild-type for Bmp5<se> must have all three represented in the pool of animals |
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