Mice that are homozygous for the Tg(SMN2)89 transgene and the Smn1 null mutation exhibit symptoms and neuropathology similar to patients with severe type I proximal spinal muscular atrophy (SMA). Homozygous mice fully congenic on the FVB/NJ genetic background (N10; Stock No. 007949) may exhibit phenotype differences compared to the incipient congenic FVB/N homozygous colony (N5; Stock No. 005024).
IMR Colony, The Jackson Laboratory
Genetic Background | Generation |
---|---|
|
Allele Type | Gene Symbol | Gene Name |
---|---|---|
Targeted (Reporter, Null/Knockout) | Smn1 | survival motor neuron 1 |
Allele Type |
---|
Transgenic (Hypomorph, Inserted expressed sequence, Humanized sequence) |
FVB.SMN2;Smn- mice are available with different amounts of backcrossing onto the FVB/N genetic background: FVB/NJ fully congenic FVB.SMN2;Smn- mice are backcrossed ten generations (N10; Stock No. 007949) while incipient congenic FVB.SMN2;Smn- mice are backcrossed five generations (N5; Stock No. 005024).
FVB/N-congenic mice homozygous for the Tg(SMN2)89 transgene (SMN2+/+) and homozygous for the Smn1tm1Msd mutation (Smn-/-) are a severe type I SMA mouse model. Homozygous mice fully congenic on the FVB/N genetic background (N10; Stock No. 007949) may exhibit phenotype differences compared to the incipient congenic FVB/N homozygous colony (N5; Stock No. 005024). The phenotype differences between these two strains, if any, have not yet been completely characterized (June 2013). Also note, Stock No. 005024 does not survive as hemizygous for the Tg(SMN2)89 transgene (SMN2+/-) and homozygous for the Smn1tm1Msd mutation (Smn-/-).
These B6.SMN2;Smn- mice harbor the Tg(SMN2)89 transgene and the Smn1 null allele (Smn1tm1Msd). The creation of the transgene and targeted mutation are described for the severe type I SMA mouse model on an incipient FVB/N congenic background; Stock No. 005024. Note the Tg(SMN2)89 transgene insertion site in on chromosome 6. FVB.SMN2;Smn- mice that had been backcrossed onto FVB/N for five generations (N5; Stock No. 005024) were obtained and then backcrossed onto FVB/NJ for additional generations. The strain after ten generations of backcrossing is FVB/NJ fully congenic FVB.SMN2;Smn- (N10; Stock No. 007949).
Expressed Gene | lacZ, beta-galactosidase, E. coli |
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Site of Expression | The expression of the lacZ gene in tissues where Smn is normally expressed was noted. |
Expressed Gene | SMN2, survival of motor neuron 2, centromeric, human |
Site of Expression | Grm7Tg(SMN2)89Ahmb |
Allele Name | targeted mutation 1, Michael Sendtner |
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Allele Type | Targeted (Reporter, Null/Knockout) |
Allele Synonym(s) | SMN- |
Gene Symbol and Name | Smn1, survival motor neuron 1 |
Gene Synonym(s) | |
Expressed Gene | lacZ, beta-galactosidase, E. coli |
Site of Expression | The expression of the lacZ gene in tissues where Smn is normally expressed was noted. |
Strain of Origin | 129P2/OlaHsd |
Chromosome | 13 |
Molecular Note | A lacZ-neo cassette was inserted into exon 2 by homologous recombination resulting in an in-frame fusion of lacZ to exon 2. Homozygous mutant embryos were identified up to 80 hours post coitum. The expression of the lacZ gene in tissues where Smn is normally expressed was noted. |
Mutations Made By | Michael Sendtner |
Allele Name | transgene insertion 89, Arthur H M Burghes |
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Allele Type | Transgenic (Hypomorph, Inserted expressed sequence, Humanized sequence) |
Allele Synonym(s) | SMN2; Tg(SMN2)89Ahmb |
Gene Symbol and Name | Grm7, glutamate receptor, metabotropic 7 |
Gene Synonym(s) | |
Promoter | SMN2, survival of motor neuron 2, centromeric, human |
Expressed Gene | SMN2, survival of motor neuron 2, centromeric, human |
Site of Expression | Grm7Tg(SMN2)89Ahmb |
Strain of Origin | FVB/N |
Chromosome | 6 |
Molecular Note | A 35.5 kb genomic fragment containing the human survival motor neuron 2 (SMN2) gene and promoter was used for the transgene. The transgene is ubiquitously expressed in all tissues examined by Northern blot analysis. Line 89 carries 1 copy of the transgene integrated into intron 4 of the gene. RT-PCR confirmed reduced expression of the gene the transgene is integrated into. |
Mutations Made By | Arthur Burghes, The Ohio State University |
The Tg(SMN2)89 transgene on chromosome 6 and the Smn1 null targeted mutation (Smn1tm1Msd) on chromosome 13 are not linked and will segregate independently. Breeding pairs offered by The Jackson Laboratory Repository are homozygous for the transgene and heterozygous for Smn1tm1Msd. These breeding pairs are phenotypically normal and do not exhibit symptoms of neuropathology. Offspring resulting from the mating of breeder pairs can possess the following genotypes:
1. Homozygous for the transgene and homozygous for the targeted mutation (25%)
2. Homozygous for the transgene and heterozygous for the targeted mutation (50%)
3. Homozygous for the transgene and wildtype at the Smn1 locus (25%)
Mice that are homozygous for the transgene and homozygous for the targeted mutation will display the SMA-like phenotype. Mice homozygous for the transgene and heterozygous for the targeted mutation will not display the SMA-like phenotype but can be mated with each other to generate additional affected mice. Mice homozygous for the transgene and wildtype at the Smn1 locus will also not exhibit an SMA-like phenotype but can be employed as control mice depending on the nature of the experiment. The Jackson Laboratory Repository also distributes mice that are homozygous for the transgene and wildtype at the Smn1 locus.
Also note, Stock No. 005024 does not survive as hemizygous for the Tg(SMN2)89 transgene (SMN2+/-) and homozygous for the Smn1tm1Msd mutation (Smn-/-).
When using the FVB.SMN2;Smn- , Severe Type I SMA , Burghes' Severe Model FVB/NJ congenic mouse strain in a publication, please cite the originating article(s) and include JAX stock #007949 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
---|---|---|
Hemizygous for Tg(SMN2)89Ahmb, Heterozygous or Wildtype for Ahmb Smn1<tm1Msd> |
Frozen Mouse Embryo | FVB.Cg-Grm7<Tg(SMN2)89Ahmb> Smn1<tm1Msd>/2J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | FVB.Cg-Grm7<Tg(SMN2)89Ahmb> Smn1<tm1Msd>/2J Frozen Embryos | $2595.00 |
Frozen Mouse Embryo | FVB.Cg-Grm7<Tg(SMN2)89Ahmb> Smn1<tm1Msd>/2J Frozen Embryos | $3373.50 |
Frozen Mouse Embryo | FVB.Cg-Grm7<Tg(SMN2)89Ahmb> Smn1<tm1Msd>/2J Frozen Embryos | $3373.50 |
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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. We do not guarantee breeding performance and therefore suggest that investigators order more than one breeding pair to avoid delays in their research.
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