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Phenotypes Associated with This Genotype
Genotype
MGI:3815033
cx26
Allelic
Composition
Bbc3tm1Ast/Bbc3tm1Ast
Tg(SOD1*G93A)1Gur/0
Genetic
Background
involves: C57BL/6 * NZB * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Bbc3tm1Ast mutation (1 available); any Bbc3 mutation (19 available)
Tg(SOD1*G93A)1Gur mutation (4 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• despite the delay in disease progression, lifespan is similar to transgenic mice wild type for Bbc3

nervous system
• gliosis and microglial activation in the spinal cord at 90 days of age are reduced compared to transgenic mice wild type for Bbc3
• however, by the end stage of the disease, gliosis and microglial activation are similar to transgenic mice wild type for Bbc3
• significant delay in motor neuron loss compared to transgenic mice wild type for Bbc3
• however, in older mice with end stage disease motor neuron loss in not different from transgenic mice wild type for Bbc3

behavior/neurological
N
• unlike transgenic mice wild type for Bbc3, no decrease in stride length is seen even at 120 days of age
• decline in paw grip endurance beginning at 98 days of age compared to 77 days of age in transgenic mice wild type for Bbc3

growth/size/body
• body weight begins to significantly decrease at 105 days of age compared to 77 days of age in transgenic mice wild type for Bbc3


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
12/17/2024
MGI 6.24
The Jackson Laboratory