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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(CAG-dsRed2/RNAi:Tardbp)6Zxu
transgene insertion 6, Zuoshang Xu
MGI:5559582
Summary 1 genotype
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(CAG-dsRed2/RNAi:Tardbp)6Zxu/0 involves: C57BL/6 * C57BL/6J * FVB/NJ * SJL MGI:5563078


Genotype
MGI:5563078
tg1
Allelic
Composition
Tg(CAG-dsRed2/RNAi:Tardbp)6Zxu/0
Genetic
Background
involves: C57BL/6 * C57BL/6J * FVB/NJ * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(CAG-dsRed2/RNAi:Tardbp)6Zxu mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 17.4% of males, and no females, have a short lifespan and die with hindlimb paralysis at a mean of 38.9 days
• 25% of males and 15.6% of females show sudden death without paralysis with a mean of 33.6 days and 41.3 days in females and males, respectively
• 57.6% of males and 84.4% of females survive longer (mean of 80-83 days) and die with whole body paralysis

behavior/neurological
• discoordinated movements
• unsteady gait
• mice show reduced vertical behaviors by 5 weeks of age
• after 10 weeks of age, mice are less active as they become progressively weaker
• mice exhibit hyperactivity beginning at 4 weeks of age, which peaks at around 5 weeks of age and ends around 10 weeks of age
• mice surviving the longest develop whole-body paralysis; this is seen in both males and females
• 17.4% of males, and no females, have a short lifespan and die with hindlimb paralysis

cardiovascular system

growth/size/body
• mice start to lose weight around 5 weeks of age

hematopoietic system
• abundant microgliosis in the ventral horn gray matter

immune system
• abundant microgliosis in the ventral horn gray matter

muscle
• necrotic skeletal muscle fibers and fibers with centralized nuclei in some areas of the muscle
• necrotic skeletal muscle fibers and fibers with centralized nuclei in some areas of the muscle
• electromyography indicates that mice develop progressive active denervation of muscles as indicated by the presence of positive sharp waves

nervous system
• abundant microgliosis in the ventral horn gray matter
• astrogliosis in the deep layer of the frontal cortex
• abundant astrogliosis in the ventral horn gray matter
• neurodegeneration in layer V cortex and ventral horn spinal cord
• dramatic motor neuron loss, with about 60% fewer motor neurons in the ventral horn
• degeneration of axons in the ventral but not dorsal roots

cellular
• necrotic skeletal muscle fibers and fibers with centralized nuclei in some areas of the muscle

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
amyotrophic lateral sclerosis type 10 DOID:0060201 OMIM:612069
J:207750





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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
03/12/2024
MGI 6.23
The Jackson Laboratory