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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1*G37R)29Dpr
transgene insertion 29, Donald L Price
MGI:3574016
Summary 4 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
cx1
Tg(NEFH)200Jpj/0
Tg(SOD1*G37R)29Dpr/0
involves: C3H/HeJ * C57BL/6 * C57BL/6J MGI:4947056
cx2
Tg(NEFH)398Jpj/0
Tg(SOD1*G37R)29Dpr/0
involves: C3H/HeJ * C57BL/6 * C57BL/6J MGI:4947061
tg3
Tg(SOD1*G37R)29Dpr/0 involves: C3H/HeJ * C57BL/6 * C57BL/6J MGI:4947055
tg4
Tg(SOD1*G37R)29Dpr/0 involves: C3H/HeJ * C57BL/6J MGI:3814057


Genotype
MGI:4947056
cx1
Allelic
Composition
Tg(NEFH)200Jpj/0
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(NEFH)200Jpj mutation (0 available)
Tg(SOD1*G37R)29Dpr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• extended mean longevity by about 6 months, compared with Tg(SOD1*G37R)29Dpr/0 mice
• all the mice are still alive after 1 year
• average lifespan is 15.8 months

behavior/neurological
• delayed onset of paralysis
• occur shortly before death with a period of about 2 weeks duration

nervous system
• axonal atrophy
• prominent perikaryal neurofilamentous accumulations
• no massive axonal loss and cell death in the motor axons in one-year old mice
• axonal atrophy
• prominent perikaryal neurofilamentous accumulations
• no massive axonal loss and cell death in the sensory axons in one-year old mice




Genotype
MGI:4947061
cx2
Allelic
Composition
Tg(NEFH)398Jpj/0
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(NEFH)398Jpj mutation (0 available)
Tg(SOD1*G37R)29Dpr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• extended mean longevity by about 2 months, compared with Tg(SOD1*G37R)29Dpr/0 mice

nervous system
• less axonal loss and cell death in the motor axons in one-year old mice, compared with Tg(SOD1*G37R)29Dpr/0 mice
• less axonal loss and cell death in the sensory axons in one-year old mice, compared with Tg(SOD1*G37R)29Dpr/0 mice




Genotype
MGI:4947055
tg3
Allelic
Composition
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6 * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G37R)29Dpr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• mean life expectancy is 9.5 months (J:69179)
• most of the mice (75%) die before 1 year old (J:69179)
• mean life span is 50.5 +/- 2.6 weeks (J:144848)
• increased mean life span and more surviving neurons after injection of lipophilic iron chelator SIH intraperitoneally (J:144848)

nervous system
• massive axonal loss and cell death in the motor axons in one-year old mice
• massive axonal loss and cell death in the sensory axons in one-year old mice

growth/size/body
• body weight drop between 47.5 and 50 weeks of age
• no changes in body weight when treated twice a week with SIH during this period

homeostasis/metabolism
• increased mitochondrial ferritin in the ventral horn motor neurons and astrocytes in the spinal cord at 12 months of age
• iron accumulation (56% increase) in the spinal cord at 12 months of age
• small, round cytoplasmic inclusions in the cell bodies of the large ventral horn motor neurons
• a more diffuse accumulation in the entire cell body in some glial cells in the gray and white matter




Genotype
MGI:3814057
tg4
Allelic
Composition
Tg(SOD1*G37R)29Dpr/0
Genetic
Background
involves: C3H/HeJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(SOD1*G37R)29Dpr mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• numerous ubiquitin-immunoreactive, Thioflavin-S positive fibrillar protein aggregates resembling hyaline inclusions are seen in the spinal cord, ventral midbrain, and the brain stem, with fewer in the cerebellum
• at late stages
• motor neuron degeneration is observed in the ventral horns of the lumbar, thoracic, and cervical spinal cord as well as the brain stem
• degeneration is associated with vacuole formation in both dendrites and axons of motor neurons

behavior/neurological
• at 6 to 8 months of age, mice exhibit axial tremors
• at 6 to 8 months of age, mice exhibit asymmetric weakness of the limbs and when suspended by their tail exhibit difficulties extending and moving hindlimbs
• at 6 to 8 months of age, mice exhibit decreased spontaneous movement compared to wild-type mice
• eventually mice develop hindlimb paralysis

muscle
• at 6 to 8 months of age, mice exhibit muscle wasting particularly along the flanks
• mice exhibit spontaneous, positive sharp waves associated with denervation atrophy

cellular
• motor neurons exhibit mitochondrial degradation

growth/size/body
• progressive

integument
• at 6 to 8 months of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:69178 , J:119631





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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory