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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID
Tg(SOD1*G93A)2Gur
transgene insertion 2, Mark E Gurney
MGI:2183718
Summary 2 genotypes
Jump to Allelic Composition Genetic Background Genotype ID
tg1
Tg(SOD1*G93A)2Gur/0 involves: C57BL/6 * FVB/N * SJL MGI:4822325
tg2
Tg(SOD1*G93A)2Gur/0 involves: C57BL/6 * SJL MGI:2446420


Genotype
MGI:4822325
tg1
Allelic
Composition
Tg(SOD1*G93A)2Gur/0
Genetic
Background
involves: C57BL/6 * FVB/N * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
cardiovascular system
• heart rate reduction
• more premature ventricular contractions

nervous system
• increased parasympathetic activity
• high frequency of atrioventricular block and premature ventricular contractions

homeostasis/metabolism
• decreased acetylcholinesterase and butyryl cholinesterase activities in the hearts




Genotype
MGI:2446420
tg2
Allelic
Composition
Tg(SOD1*G93A)2Gur/0
Genetic
Background
involves: C57BL/6 * SJL
Find Mice Using the International Mouse Strain Resource (IMSR)
No mouse lines available in IMSR.
See publication links below for author information.
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• animals are moribund by 5 months of age

growth/size/body
• weight loss starting around 13 weeks of age

behavior/neurological
• impaired grooming evident at 3 to 4 months
• mutants extend their hind legs less than normal when lifted by the tail
• develop a tremor of the hindlimbs when suspended in the air (J:32665)
• fine hind limb tremors starting around 12 weeks (J:89928)
• consistently worse performance than the control animals on the rotarod
• decline steadily after 15 weeks of age for the rotarod, around 12 weeks for the hanging wire
• unable to remain on the rotarod at 19 weeks of age, or 20 weeks for the hanging wire
• the hanging wire test has a higher diagnostic accuracy
• normal limb posture when not moving, however after initiation of movement, hindlimbs and toes frequently lock in a hyperextended position
• 4mutants become paralyzed in one or more limbs after 5 months of age

muscle
• hindlimb weakness evident at 3 to 4 months

nervous system
• intraneuronal vacuoles and vacuoles in the surrounding neuropil
• intraneuronal vacuoles and vacuoles in the surrounding neuropil in the brainstem
• swollen axons with a dense or vacuolated axoplasm at 163 days of age
• irregular myelin sheeths in contour
• irregular frayed myelin sheeths
• decreased numbers of axons in anterior root at 113 days of age
• swollen axons, with a dense or vacuolated axoplasm
• irregular myelin sheeths in contour
• irregular frayed myelin sheeths
• macrophages with myelin debris
• exquisite localization of vacuoles to the cytoplasm of the large motor neurons of the spinal anterior horns, particularly in the lateral portions at 73 days of age
• vacuoles inside the neuronal cell bodies and in the surrounding neuropil at 163 days of age
• loss of choline acetyltransferase-containing spinal motor neurons, particularly in the ventral spinal cord (J:32665)
• decreased size of the anterior horns at 180 days of age (J:78629)
• severe loss of motor neurons (J:78629)
• surviving neurons are atropic with an irregular contour and eosinophilic hyaline inclusions (J:78629)
• exquisite localization of vacuoles to the cytoplasm of the large motor neurons of the spinal anterior horns, particularly in the lateral portions at 73 days of age
• vacuoles inside the neuronal cell bodies and in the surrounding neuropil at 163 days of age
• decreased size of the anterior horns at 180 days of age
• swollen axons, some with axoplasmic vesicles in their course from the anterior horn to the anterior root at 163 days of age
• mild swelling nerve fibers in the intermediate zone of the posterior columns and posterior roots
• swollen axons, with a dense or vacuolated axoplasm in lateral columns at 180 days of age
• irregular frayed myelin sheeths
• vacuolation of axoplasm at 163 days of age
• axonal swellings with a dense axoplasmic appearance
• irregular myelin sheeths in contour
• irregular frayed myelin sheeths
• swollen axons, with a dense or vacuolated axoplasm in anterior columns at 180 days of age
• loss of large myelinated axons in intramuscular nerves and from ventral motor roots
• rarely greatly expanded axonal profiles pack with dense fibrillary

cellular
• vacuoles in the rough endoplasmic reticulum
• vacuoles in the mitochondrion
• large vacuoles cause linearization of the entire structure

integument
• coats develop a coarse appearance

Mouse Models of Human Disease
OMIM ID Ref(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:32665 , J:78629 , J:89928





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last database update
06/22/2016
MGI 6.04
The Jackson Laboratory