These Aga knock-out mice exhibit normal early development which slowly progresses to severe mental and motor retardation in early adulthood. They are suitable for use in applications related to the study of the human lysosomal disease aspartylglycosaminuria.
Dr. Vesa Kaartinen, University of Michigan
Genetic Background | Generation |
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|
Allele Type | Gene Symbol | Gene Name |
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Targeted (Null/Knockout) | Aga | aspartylglucosaminidase |
Mice with a null Aga gene, which encodes the lysosomal enzyme glycosylasparaginase, exhibit normal early development which slowly progresses to severe mental and motor retardation in early adulthood. At 5-10 months of age, massive accumulation of glycoaspartylglucosamine can be detected along with lysosomal vacuolization, axonal swelling in the gracile nucleus and impaired neuromotor coordination. After 10 months of age, there is progressive motor impairment and impaired bladder function and premature death. The mutant mice loose fertility earlier than the wild type siblings.
Allele Name | targeted mutation 1, Vesa Kaartinen |
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Allele Type | Targeted (Null/Knockout) |
Allele Synonym(s) | Aga-; AGU |
Gene Symbol and Name | Aga, aspartylglucosaminidase |
Gene Synonym(s) | |
Strain of Origin | (129X1/SvJ x 129S1/Sv)F1-Kitl+ |
Chromosome | 8 |
Molecular Note | A neomycin selection cassette was inserted into the coding sequences in exon 3. Northern blot analysis and RT-PCR demonstrated that no detectable transcript is produced from this allele. Enzyme activity assays on liver and brain extracts of homozygous mice confirmed that no functional protein is made from this allele. |
Mutations Made By | Dr. Vesa Kaartinen, University of Michigan |
When maintaining a live colony, these mice can be bred as homozygotes.
The expected coat colors from breeding are white bellied agouti, and yellow with pink eyes.
When using the Aga- mouse strain in a publication, please cite the originating article(s) and include JAX stock #003463 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Heterozygous or wildtype for Aga<tm1Vk> |
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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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