These transgenic mice express the constitutively active point mutation, SmoA1, of the mouse smoothened homolog (Drosophila) (Smo) gene, under the control of the mouse neurogenic differentiation 2 (Neurod2) promoter, with transgene expression specific to cerebellar granule cells. Homozygous mice show an increased medulloblastoma incidence. This transgenic mouse strain may be useful in studies of medulloblastoma and metastasis.
James M Olson, Fred Hutchinson Cancer Research Center
These Smo/Smo transgenic mice express the constitutively active point mutation, SmoA1, of the mouse smoothened homolog (Drosophila) (Smo) gene, under the control of the 1kb mouse neurogenic differentiation 2 (Neurod2) promoter. Transgene expression is specific to cerebellar granule cells, as observed by His tag staining. Tumor incidence in mice homozygous for the transgene is 85% by one month of age and 94% by two months of age, with an average age of onset of related traits being four months. Traits associated with advanced tumors include enlarged posterior fossa, and/or tilted head, and hunched posture. Weight loss and an ungroomed appearance are also common. The disease progresses to leptomeningeal metastasis of the brain and spine. Once clinical symptoms are noted, survival is one to two weeks. This mutant mouse strain may be useful in studies of medulloblastoma and metastasis.
The ND2:SmoA1 transgene containing the entire mouse smoothened homolog (Drosophila) (Smo) gene, with the constitutively active point mutation SmoA1, driven by the 1kb mouse neurogenic differentiation 2 (Neurod2) promoter was microinjected into C57BL/6. Founder line A1.199 was subsequently established. The hemizygote animals were were backcrossed to both C57BL/6J and C57BL/6NCrl for an unknown number of generations. The hemizygotes were then intercrossed to produce homozygotes, referred to as Smo/Smo mice. The mice were maintained as homozygotes for more than 10 generations before arriving at The Jackson Laboratory. The transgene insertion site has been mapped by plasmid probe to Chromosome 14 at band qC1.
|Expressed Gene||Smo, smoothened, frizzled class receptor, mouse, laboratory|
|Site of Expression|
|Allele Name||transgene insertion 199, James M Olson|
|Allele Type||Transgenic (Constitutively active, Inserted expressed sequence)|
|Allele Synonym(s)||ND2:SmoA1; SmoA1; SmoA1:199|
|Gene Symbol and Name||Tg(Neurod2-Smo*A1)199Jols, transgene insertion 199, James M Olson|
|Gene Synonym(s)||ND2:SmoA1; ND2:SmoA2; SmoA1:199|
|Promoter||Neurod2, neurogenic differentiation 2, mouse, laboratory|
|Expressed Gene||Smo, smoothened, frizzled class receptor, mouse, laboratory|
|Strain of Origin||C57BL/6|
When maintaining a live colony, these mice can be bred as homozygotes. However, subclinical tumor incidence in homozygotes is 85% by 1 month of age and 94% by 2 months of age. The average age of onset of clinical tumor symptoms at four months. Once clinical symptoms are noted, survival is 1-2 weeks. It is not uncommon for female breeders to develop a tumor while pregnant or nursing. When this occurs, pups are fostered into another age-matched litter. The Donating Investigator reports that some breeder pairs will produce only one litter while most will have three litters before tumor formation.
The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided,
their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of
each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders
are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in
order to provide the minimum number of animals, animals will ship within 25 weeks.
The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.
Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation.
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The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
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