These mice carry a spontaneous mutation at the Lama2 locus characterized by progressive weakness and paralysis.
Read More +Genetic Background | Generation |
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000664 C57BL/6J |
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Allele Type | Gene Symbol | Gene Name |
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Spontaneous | Lama2 | laminin, alpha 2 |
Mice homozygous for the dystrophia-muscularis spontaneous mutations (Lama2dy and Lama2dy-2J) are characterized by progressive weakness and paralysis beginning at about 3 1/2 weeks of age. The hindlimbs are affected first, later the axial and forelimb musculature. Death usually occurs before 6 months of age, and mutant mice are usually sterile. Skeletal muscle shows degenerative changes with proliferation of sarcolemmal nuclei, increase in amount of interstitial tissue, and size variation among individual muscle fibers. In the dorsal and ventral roots of the peripheral nerves, both spinal and cranial, Schwann cells fail to separate and ensheathe axons so that groups of closely apposed naked axons, normally seen only in early stages, persist into adulthood. In the rest of the PNS, the basement membrane of Schwann cells is interrupted by gaps, the internodal gap in the nodes of Ranvier is lengthened, and there is delayed onset of myelination with fewer myelinated axons and shorter internode length. Homozygotes are usually sterile.
In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. This is the case for the strain above. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.
Allele Name | dystrophia muscularis |
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Allele Type | Spontaneous |
Allele Synonym(s) | dy |
Gene Symbol and Name | Lama2, laminin, alpha 2 |
Gene Synonym(s) | |
Strain of Origin | 129P1/Re |
Chromosome | 10 |
General Note | This mutation arose spontaneously in the 129/Re inbred strain at The Jackson Laboratory in 1951. |
Molecular Note | Analysis of Lama2 expression of dystrophic dy mice revealed a specific deficiency of this mRNA in skeletal muscle, cardiac muscle, and peripheral nerve. |
When maintaining a live colony, heterozygous mice may be bred to wildtype siblings, or to C57BL/6J inbred mice (Stock No. 000664). Homozygotes are usually sterile.
When using the dystrophia muscularis mouse strain in a publication, please cite the originating article(s) and include JAX stock #000631 in your Materials and Methods section.
Facility Barrier Level Descriptions
Service/Product | Description | Price |
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Progeny testing required but not provided. No genotyping assay is available for these recessive cryo-recovered animals of undefined genotype |
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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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