Overview

Also Known As:B6.SOD1-G93A

These SOD1-G93A transgenic mice express a G93A mutant form of human SOD1 and may be useful in studying neuromuscular disorders such as Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).
Our preclinical efficacy testing services offer scientific expertise and an array of target-based and phenotype-based outcome measures, both in vivo and at endpoint, for flexible study designs and assay development in mouse models of Amyotrophic Lateral Sclerosis. See our full service platform.

Donating Investigator

Gregory Cox, The Jackson Laboratory

Genetic overview

Genetic Background	Generation
	N28+N45 (2019-06-14 00:00:00)

Tg(SOD1*G93A)1Gur

Allele Type
Transgenic (Inserted expressed sequence, Humanized sequence)

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Research Applications

Metabolism Research
Neurobiology Research
Mouse/Human Gene Homologs

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Base Price

Starting at:

$270.00 Domestic price for female 4-week

296.83 Domestic price for breeder pair

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Details

Detailed Description

Mice hemizygous for this SOD1-G93A (also called G93A-SOD1) transgene are viable and fertile, with transgenic expression of a G93A mutant form of human SOD1. This founder line (often referred to as G1H) is reported to have high transgene copy number. Hemizygotes exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans; becoming paralyzed in one or more limbs with paralysis due to loss of motor neurons from the spinal cord. Motor neuron degeneration has been associated with function and/or degeneration of astrocytes, the major glial cell type of the nervous system.
Transgenic mice have an abbreviated life span: 50% survive at 157.1+/-9.3 days (in contrast to the mixed B6SJL background where 50% survival is observed at 128.9+/-9.1 days).
Female hemizygotes are poor breeders, and rarely produce more than one litter before the onset of disease. In contrast to LPS-induced microglia and activated M1/M2 macrophages, spinal cord microglia activated by disease progression do not upregulate genes that display a bias to either an M1 (neurotoxic) phenotype or an M2 (protective) phenotype. The pattern of gene expression in SOD1^G93A activated microglia represents a unique ALS-specific signature. These SOD1-G93A (also called G93A-SOD1) transgenic mice may be useful in studying neuromuscular disorders, including Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig's Disease).

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. As the SOD1-G93A transgenic mice were originally created on a mixed genetic background, it should be noted that the phenotype of the congenic mice could vary from that originally described. We will modify the strain description if necessary as published results become available.

Development

The SOD1-G93A (or G93A-SOD1) transgene was designed with a mutant human SOD1 gene (harboring a single amino acid substitution of glycine to alanine at codon 93) driven by its endogenous human SOD1 promoter. This transgene was injected into fertilized B6SJLF1 mouse eggs and founder animals were obtained. Transgenic mice on a mixed B6SJL genetic background were sent to The Jackson Laboratory (as Stock No. 002726). Upon arrival, some mice were backcrossed to C57BL/6J for at least 10 generations to generate this congenic strain (Stock No. 004435). The backcross was completed in July 2002.

Expression Data

Expressed Gene	SOD1, superoxide dismutase 1, human
Site of Expression

Control Suggestions

Noncarrier
000664 C57BL/6J

Additional Information

Considerations for Choosing Controls

Selected References

Gurney ME; Pu H; Chiu AY; Dal Canto MC; Polchow CY; Alexander DD; Caliendo J; Hentati A; Kwon YW; Deng HX; Chen W; Zhai P; Sufit RL; Siddique T. 1994. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149] Science 264(5166):1772-5PubMed: 8209258 MGI: J:32665
Tu PH; Raju P; Robinson KA; Gurney ME; Trojanowski JQ; Lee VM. 1996. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A 93(7):3155-60PubMed: 8610185 MGI: J:76718
Wooley CM; Sher RB; Kale A; Frankel WN; Cox GA; Seburn KL. 2005. Gait analysis detects early changes in transgenic SOD1(G93A) mice. Muscle Nerve 32(1):43-50PubMed: 15880561 MGI: J:115355

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Genetics

Tg(SOD1*G93A)1Gur

Allele Symbol: Tg(SOD1*G93A)1Gur

Allele Name	transgene insertion 1, Mark E Gurney
Allele Type	Transgenic (Inserted expressed sequence, Humanized sequence)
Allele Synonym(s)	(G93A)Tg+; G1H; G93A; G93A SOD1; G93A+; G93AGur^dl; G93A-SOD1; Gur1-G93A; hSOD1G93A; SOD1 G93A; SOD1 Tg; SOD1^G93A; Tg(G93A-SOD1)1Gur; Tg(SOD1-G93A)1Gur; TgN(SOD1-G93A)1Gur; TgN[SOD1-G93A]1Gur
Gene Symbol and Name	Tg(SOD1*G93A)1Gur, transgene insertion 1, Mark E Gurney
Gene Synonym(s)
Promoter	SOD1, superoxide dismutase 1, human
Expressed Gene	SOD1, superoxide dismutase 1, human
Strain of Origin	(C57BL/6 x SJL)F1
Chromosome	12
General Note	This line, G1H, was derived from the original G1 line (now designated Tg(SOD1*G93A)2Gur) reported in J:32665. Transgenic mice on a background that involves C57BL/6 and SJL express high levels of the transgene with a 4-fold increase in SOD activity, and exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans. Hemizygous transgenic mice become paralyzed in one or more limbs and have a life span of approximately 19-23 weeks. Paralysis is due to loss of motor neurons from the spinal cord.
Molecular Note	This transgenic subline (designated G1H in J:76718) is derived from the G1 parental transgenic line (originally described in J:32665). This line carries a 40% expansion in transgene copy number compared to the original G1 line (described in J:32665, in MGI as Tg(SOD1*G93A)2Gur). The transgene construct is composed of the human SOD1 gene carrying a glycine to alanine transition at position 93 (G93A). The G93A mutation does not alter the activity of the protein. This line carries a high copy number maps to Mus Chr12:97,165,800 (coordinates from MGSC ver 37, mm9).
Mutations Made By	Dr. Mark Gurney, Tetra Discovery Partners

Disease/Phenotype

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

amyotrophic lateral sclerosis type 1

Research Areas By Phenotype

This mouse can be used to support research in many areas including:

Metabolism Research
Neurobiology Research
- Metabolic Defects
- Neurodegeneration
- Amyotrophic Lateral Sclerosis (ALS)
Mouse/Human Gene Homologs
- amyotrophic lateral sclerosis (ALS)

Mammalian Phenotype Terms by Genotype

The following phenotype information is associated with a similar, but not exact match to this JAX^® Mice strain

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6 SJL

adipose tissue phenotype

abnormal adipose tissue morphology
- exhibit reduced adipose tissue accumulation

abnormal fat pad morphology
- epididymal and retroperitoneal white adipose tissue is markedly reduced or almost nonexistent in the asymptomatic phase of the disease

cellular phenotype

abnormal cell physiology
- decreased glutamate uptake in synaptosomes

decreased cellular glucose uptake
- decreased glucose uptake in synaptosomes

increased cellular sensitivity to oxidative stress
- increased basal and induced lipid peroxidation levels in synaptosomes

growth/size/body region phenotype

decreased body weight
- lower body mass

weight loss
- body weight begins to significantly decrease at 77 days of age

homeostasis/metabolism phenotype

abnormal circulating hormone level

abnormal energy expenditure
- exhibit increased energy expenditure at rest

abnormal nitric oxide homeostasis
- decreased spinal cord nNOS activity at 165 days of age
- increased spinal cord iNOS activity at 117 and 165 days of age

decreased circulating insulin level

decreased circulating leptin level
- plasma leptin levels are diminished

increased cerebral infarction size
- increased infarct volume at 24 hours after transient focal cerebral ischemia

increased circulating corticosterone level

increased oxygen consumption
- exhibit higher rates of total oxygen consumption

behavior/neurological phenotype

abnormal active avoidance behavior
- less conditioned responses
- less increased performance as training proceeds
- normal minimal foot-shock intensity eliciting vocalization

abnormal gait
- impairment in walking patterns with reduced stride length beginning at 90 days of age

abnormal locomotor behavior
- locomotor activity decreases more rapidly across 5 sessions

abnormal motor capabilities/coordination/movement
- the first signs of motor neuron disease, hyperflexia, crossed spread of spinal reflexes, and shaking of the limbs when suspended in the air, occur by 91 days of age

decreased grip strength
- steady decline in paw grip endurance beginning at 77 days of age

hindlimb paralysis
- develop a progressive worsening paresis involving primarily the hind limbs with atrophy of the skeletal musculature

impaired coordination
- unable to stay for 2 min on the rotarod beyond 118 ¿ 4 days of age

paralysis
- develop paralysis rapidly after signs of hindlimb weakness
- end-stage disease occurs at an average of 136 days, with mutants exhibiting severe paralysis and inability to forge for food or water

short stride length
- beginning at 90 days of age

mortality/aging

premature death
- die after 124 ¿ 4 days
- die after 124 ± 4 days
- die within 7-10 days after paralysis
- Background Sensitivity - increased survival on C57BL/6J background (50% survival at 157.1+/-9.3 days) in contrast to B6SJL background (50% survival at 128.9+/-9.1 days)

muscle phenotype

abnormal muscle physiology
- mice exhibit decreased muscle tetanic force compared with wild-type mice
- mice exhibit reduced survival of motor units compared with wild-type mice
- the extensor digitorum longus exhibits fatigue resistance compared with wild-type muscles

muscle weakness
- hindlimb weakness from 4 months of age
- severe muscle weakness beyond 3 months of age

skeletal muscle atrophy
- develop atrophy of skeletal musculature

nervous system phenotype

abnormal CNS synaptic transmission

abnormal excitatory postsynaptic potential
- intracellular recordings of single excitatory postsynaptic potentials from striatal medium spiny neurons showing an LTP, instead of an LTD

abnormal long term potentiation
- a tetanic stimulation induced a long term potential (LTP), instead of an long term depression (LTD) in field potentials induced in the striatum following corticostriatal pathway stimulation

abnormal motor neuron morphology
- mutant SOD1-positive inclusions in the ventral horn starting at P30
- the number of motoneurons with aggregates decreased dramatically over time

abnormal nervous system physiology
- mutants develop amyotrophic lateral sclerosis-like disease, with onset of disease on average at 103.2 days

abnormal neuron physiology
- increased expression of unfolded protein response target genes by 50 days of age in motor neurons

abnormal paired-pulse inhibition
- trend towards less susceptible to paired-pulse depression

abnormal spinal cord morphology
- exhibit neurofilament-rich spheroids at 82-days of age, similar to those seen in human amyotrophic lateral sclerosis; other neuronal intermediate filament proteins (alpha-internexin, peripherin) also accumulate in the spheroids
- exhibit thickened dystrophic neurites filled with immunoreactive neurofilament-rich inclusions
- more spheroids are seen in cervical and thoracic regions compared to lumbar and sacral spinal cord in early symptomatic mutants, however similar numbers at all spinal cord levels are seen in older mutants
- spheroids are more frequently found in the anterior horn and in the anterior and lateral columns of the white matter than in the posterior horn

astrocytosis
- exhibit astrocytosis in spinal cord, mainly in the anterior and lateral horns
- reactive astrocytes in the gray matter of the spinal cord at 117 and165 days of age, predominated in the anterior horn of the spinal cord
- resting astrocytes in the white matter of the spinal cord at both the cervical and lumbar levels
- using GFAP as a specific marker of astrocytes

decreased motor neuron number
- motoneuron loss beyond 3 months of age

gliosis
- gliosis and microglial activation are seen in the spinal cord by 90 days of age
- reactive microglial cells in the gray matter of the spinal cord at 117 and165 days of age
- resting microglial cells in the white matter of the spinal cord
- using MAC-1 and F4/80 as specific markers of microglial cells

impaired synaptic plasticity
- abnormal corticostriatal synaptic plasticity between postnatal days 100 and 110

increased cerebral infarction size
- increased infarct volume at 24 hours after transient focal cerebral ischemia

motor neuron degeneration
- develop motor neuron disease; exhibit degenerating motor neurons filled with perikaryal vacuoles in the anterior and lateral horns
- significant decrease in sciatic motor neuron survival by 90 days of age

reduced long term depression
- exogenous dopamine (DA) restores LTD
- in the presence of the N-methyl-D-aspartic acid (NMDA) receptor antagonist AP5, repetitive stimulation of the corticostriatal pathway results in LTD
- the degree of field-amplitude depression is smaller in the presence of the NMDA receptor antagonist AP5
- the presence of the selective DA D2 receptor agonist restores LTD

Genotype: Tg(SOD1G93A)1Gur/?
involves: C57BL/6 SJL

behavior/neurological phenotype

hypoactivity
- mice exhibit decreased locomotor activity compared to wild-type mice after day 115

growth/size/body region phenotype

decreased body weight
- body weights are lower than in wild-type mice and continues to deteriorate after day 115

mortality/aging

premature death
- mice reach end stage by day 147 +/-2.5 SEM (standard error of measurement)

nervous system phenotype

abnormal spinal cord morphology
- degeneration of the motor neurons in the cervical and lumbar spinal cord is visible at end stage with astrocytosis and vacuolization

astrocytosis
- astrocytosis is evident in the spinal cord

decreased motor neuron number
- 50% of motor neurons in the cervical spinal cord and 60% in lumbar spinal cord are degenerated at day 152

Genotype: Tg(SOD1G93A)1Gur/0
B6SJL-Tg(SOD1G93A)1Gur/J

behavior/neurological phenotype

abnormal drinking behavior
- progressive decrease in lick rhythm over time beginning at approximately 102 days of age

abnormal motor capabilities/coordination/movement

decreased grip strength
- progressive reduced forelimb and hindlimb grip force from the onset of testing (64 days of age)
- reduced muscle strength in this traction test even from the age of 35 days

hindlimb paralysis
- dragging in one hindlimb and inability to grasp a cage bar at 103 days of age
- first signs of hindlimb paralysis at 3.5-5 months of age
- paralysis progresses to both hindlimbs within 3-4 days

hypoactivity
- longer duration of stops at 3.5-4.5 months of age
- lower horizontal travel distance and number of stops at 3.5 months of age in open field analysis
- progressively decreased overall motor activity during the first 3 months

impaired coordination
- failed rotarod test after 113 days of age
- lower performance rate (<90%) in rotarod test from 71 days of age

increased anxiety-related response
- reduced mean time spent in the center of the open field from 4 months on
- slightly increased anxiety-related behavior

tremors
- hind limb tremors at 14 weeks of age
- mild hindlimb tremor at 90 days of age

muscle phenotype

abnormal muscle physiology
- greater osmotic stress-induced Ca²⁺ release activity in fiber segments with depolarized mitochondria

abnormal skeletal muscle fiber morphology
- enlarged mitochondria with vacuoles, invading the sarcomeric A band

muscle weakness
- progressive decrease in tongue force beginning at approximately 113 days of age
- progressive reduced forelimb and hindlimb grip force from the onset of testing (64 days of age)

progressive muscle weakness
- after 120 days of age
- dragging in one hindlimb and inability to grasp a cage bar at 103 days of age
- paralysis progresses to both hindlimbs within 3-4 days

nervous system phenotype

abnormal astrocyte morphology
- characterized by mitochondrial damage, cellular edema and cell rupture
- swollen, progressive degeneration of astrocytes in the brainstem, cervical and lumbar spinal cord

abnormal axonal transport
- decreased fraction of anterograde mitochondria
- increased number of retrograde mitochondria by around 80% after fast axonal transport (FAT)

abnormal brain morphology
- degenerating somata in the brain at 4 months of age

abnormal brainstem morphology
- continuous age-dependent increased number of vacuoles
- continuous age-dependent T2 values increase
- degeneration of brainstem motor nuclei (the trigeminal, hypoglossal and facial nucleus as early as Day 80 by magnetic resonance imaging (MRI)
- H&E staining with brain sections show same morphological alterations
- higher relaxation parameter T2 values for all the three brainstem nuclei after 80 days
- increased area of the brainstem nuclei (hypoglossal and facial nucleus) between 80 and 120 days after birth
- increased number of vacuoles in the brainstem nuclei from Day 80 onwards
- swollen neurites and vacuoles in the brainstem affecting cerebellar and various motor nuclei (the hypoglossal, the ambiguus and the facial nucleus) within the following month
- vacuoles in the trigeminal nucleus and the locus coeruleus neuropil at 3 months of age

abnormal cerebral cortex morphology
- degenerative pyramidal-shaped neurons and several long neurites in motor as well as in extra-motor areas of the cerebral cortex at 5 months of age

abnormal corticospinal tract morphology
- progressive loss of corticospinal tract neurons (9% at 60 days, 30% at 90 days and 53% at 110 days)
- progressive loss of dorsal corticospinal axons (13% at 60 days, 22% at 90 days and 32% at 110 days)

abnormal globus pallidus morphology
- degenerative changes in the globus pallidus at 5 months of age

abnormal hypothalamus morphology
- degenerative changes in the hypothalamic nuclei at 5 months of age

abnormal microglial cell morphology
- increased cell size starting at day 100
- increased granularity starting at day 100
- large vacuoles in microglial cell cytoplasm in the cervical and lumbar spinal cord

abnormal midbrain morphology
- degeneration lesions in the retrorubral field

abnormal motor neuron morphology
- decreased mitochondria in axonal mitochondria
- Normal - however, lipofuscin is detected in the neuron cell bodies of the dorsal regions of the spinal cord gray matter
- increased intermitochondrial distance by 30-50% in axons
- increased mitochondria in cell bodies of motoneurons
- large membrane bound vacuoles consisting of either dilated axons or dendrite
- mitochondria with swollen cristae in both axons and dendrites near capillaries
- more electron-lucent than electron-dense motoneurons by 18 weeks, and many motoneurons exhibit extensive vacuolation
- most ventral horn motor neurons lack autofluorescent lipofuscin in cell bodies at 3 months of age
- paler and more strongly stained motoneurons, with vacuolation in the more strongly stained motoneurons by 6 weeks

abnormal nervous system physiology
- lower basal ATP levels in cerebral cortex in 30-day-old mice
- partially ameliorated by creatine administration
- reduced ADP levels by 60 days of age
- reduced creatine levels by 90 days of age in both cerebral cortex and spinal cord
- the extent of impairment in ATP production progresses with age

abnormal oculomotor nucleus morphology
- heavily filled with Gallyas+, coiled and ballooned axonal and dendritic profiles in the oculomotor ,the trochlear nucleus at 4 months of age

abnormal red nucleus morphology
- spongio-form degenerative changes in the red nucleus

abnormal spinal cord morphology
- increase and extend into the dorsal horn of the spinal cord within the following month
- increase with disease progression, 36-fold by day 135
- increased lymphocyte population in spinal cords at day 65
- natural killer cell in spinal cords
- restricted to the ventral horns of spinal cords at the age of 2 months
- significant accumulation of CD4+ and CD8+ T cells in spinal cords
- swollen neurites and vacuoles of various dimensions and large neuritic spheroids in some spinal motor neurons at the age of 2 months

abnormal spinal cord ventral horn morphology
- most ventral horn motor neurons lack autofluorescent lipofuscin in cell bodies at 3 months of age

abnormal substantia nigra morphology
- degeneration lesions in the substantia nigra

abnormal superior colliculus morphology
- degeneration lesions in the deep layers of the colliculi superior

abnormal synaptic bouton morphology
- degenerative changes occurred in all classes of terminal at 6, 10 and 18 weeks of age, apart from the C-type terminal
- increase in the membrane coverage of C-terminals at 6, 10 and 18 weeks of age
- increase in the proportional numbers of C-terminals at 6 and 18 weeks of age
- increased length of the subsynaptic cistern of C-terminals
- increased number of the Nissl body rER lamellae of C-terminals at 18 weeks of age
- increased overall length of the Nissl body rER lamellae of C-terminals at 10 and 18 weeks of age
- increased size of the postsynaptic structure of C-terminals
- progressive enlargement of the C-terminal presynaptic terminal at 10 and 18 weeks of age
- reduction in both terminal number and coverage of the somal membrane, and decline further by around 60% at 18 weeks of age
- thin glial processes either partly or totally engulfed some terminals at 18 weeks of age

abnormal tegmentum morphology
- degeneration lesions in the neuropil of the ventral tegmental field

abnormal thalamus morphology
- degeneration lesions in the nucleus anterior thalami

astrocytosis
- appear simultaneously to the degenerative changes and increase substantially with time

brain vacuoles
- continuous age-dependent increased number of vacuoles
- increased number of vacuoles in the brainstem nuclei from Day 80 onwards
- vacuolization in the brain and reach telencephalic regions at 5 months of age

CNS inflammation
- first in the spinal cord, predominantly in the white matter
- later in the degenerating regions up to the mesencephalon
- lymphocytes and CD11c positive dendritic cells infiltrate the affected CNS regions not before 4 months of age

decreased motor neuron number
- loss of 30% of corticospinal, 33% of bulbospinal and 33% of rubrospinal neurons at 90 days
- loss of 53% of corticospinal, 41% of bulbospinal and 43% of rubrospinal neurons at 110 days
- progressive loss of upper motor neurons
- small loss of corticospinal (9%), bulbospinal (12%) and rubrospinal (14%) projections at 60 days

microgliosis
- appear simultaneously to the degenerative changes and increase substantially with time
- first noted at 6 weeks
- increase in the number of glial processes abutting onto the neuronal surface and apposed to the presynaptic terminal in 10-week-old and 18-week-old mice
- microglia (CD11b+ CD45lo) population expanded 1.65-fold by day 135 in spinal cords
- some microglial cells occupy areas where formerly occupied by astrocytes or oligodendrocytes in the cervical and lumbar spinal cord

motor neuron degeneration
- intracellular edema in some motor neurons, characterized by cytoplasmic enlargement
- swollen and degenerating motor neurons in the late stage in the brainstem, cervical and lumbar spinal cord
- vacuolization and silver-impregnated neurites at 2 months of age in the spinal cord, proceed caudocranially into the brain and reach telencephalic regions at 5 months of age

cardiovascular system phenotype

abnormal capillary morphology
- disruption of blood-brain barrier and blood-spinal cord barrier in areas of motor neuron degeneration in the brainstem, cervical and lumbar spinal cord
- intracellular edema, endoplasmic reticulum swelling and formation of numerous large vacuoles in capillary endothelial cells cytoplasm
- multiple layers of endothelial cells separated by sheets of basement membrane material
- pericytes under the capillary basement membrane

abnormal vascular endothelial cell morphology
- characterized by mitochondrial damage, swelling of endoplasmic reticulum, cytoplasmic vacuolization and cell death
- endothelial cell membrane and/or basement membrane damage, followed by vascular leakage
- progressive swollen and degenerating capillary endothelial cells in the brainstem, cervical and lumbar spinal cord

cellular phenotype

abnormal cell physiology
- decreased anterograde frequency by almost 60% for the activity of mitochondrial molecular motors
- decreased glutamate uptake in synaptosomes at 150 days of age
- decreased velocity of anterograde transport
- normal overall level of activity of mitochondrial molecular motors
- normal retrograde frequency
- normal velocity of retrograde transport
- reduced persistence of movement in the anterograde direction

abnormal endoplasmic reticulum morphology
- dilated Golgi-ER and less organized, with increased numbers of separated highly dilated ER profiles
- dilation of the endoplasmic reticulum in motor neuron cytoplasm in the brainstem, cervical and lumbar spinal cord
- Nissl body rough ERs (rER) in the terminals appear more prominent, with evidence of polyribosomal hyperplasia at 10 weeks of age
- rER damage appearing as 'splits' in the cytoplasm by 18 weeks

abnormal mitochondrial crista morphology
- reduced numbers of short, 'broken' cristae in the terminals at 10 weeks of age

abnormal mitochondrial physiology
- loss of mitochondrial inner membrane potential in fiber segments near the neuromuscular junction starting at the age of 37 days

abnormal mitochondrial shape
- lower aspect ratio showing rounding up mitochondria

abnormal mitochondrion morphology
- characterized by swelling, cristae disruption and eventual vacuolization of mitochondria
- disorganized mitochondrial cristae and degenerating mitochondria in astrocytes, capillary endothelial cells and neuropil in the brainstem, cervical and lumbar spinal cord

abnormal respiratory electron transport chain
- reduced mitochondrial electron transport activity

dilated mitochondria
- swollen mitochondria

increased mitochondria size
- enlarged mitochondria with vacuoles, invading the sarcomeric A band

growth/size/body region phenotype

slow postnatal weight gain
- no weight gain beginning at approximately 108 days of age

hematopoietic system phenotype

abnormal lymphocyte morphology
- a number of lymphocytes spontaneously form rosettes with autologous erythrocytes in two thirds of the mice

abnormal microglial cell morphology
- increased cell size starting at day 100
- increased granularity starting at day 100
- large vacuoles in microglial cell cytoplasm in the cervical and lumbar spinal cord

abnormal spleen morphology
- diminished follicular architecture with a greater number of follicles at end stage

abnormal splenic cell ratio
- decreased levels of CD45RA+ (naive) T cells
- increased levels of CD45RO+ (memory) T cells

abnormal splenocyte morphology
- increased annexin-V associated apoptosis and necrosis of lymphocytes at pre-symptomatic stage (14 weeks of age)

decreased eosinophil cell number
- decreased number of circulating eosinophils

decreased leukocyte cell number
- Normal - normal neutrophil levels
- reduced WBC numbers

decreased lymphocyte cell number
- lower lymphocyte density

decreased monocyte cell number
- decreased number of circulating monocytes

decreased spleen weight
- reduced spleen weight (45%) at 19 weeks of age

increased basophil cell number
- increased basophil number compared with Tg(SOD1)2Gur mice

microgliosis
- appear simultaneously to the degenerative changes and increase substantially with time
- first noted at 6 weeks
- increase in the number of glial processes abutting onto the neuronal surface and apposed to the presynaptic terminal in 10-week-old and 18-week-old mice
- microglia (CD11b+ CD45lo) population expanded 1.65-fold by day 135 in spinal cords
- some microglial cells occupy areas where formerly occupied by astrocytes or oligodendrocytes in the cervical and lumbar spinal cord

small spleen
- reduced spleen size at end stage (20-22 weeks of age)

homeostasis/metabolism phenotype

abnormal calcium ion homeostasis
- greater osmotic stress-induced Ca²⁺ release activity in fiber segments with depolarized mitochondria

abnormal glucose homeostasis
- components of the bulbospinal projection pathway are compromised by 60 days of age, whereas rubrospinal projections become involved later
- impaired glucose utilization rates in multiple brain components of the motor system as early as 60 days of age
- reductions in glucose use in the gray matter of cervical, thoracic, or lumbar regions of the spinal cord in 120-day-old mice

edema
- characterized by the formation of protein-filled areas in the extracellular space formerly occupied by astrocytes or neuropil
- progressive edema in the brainstem, cervical and lumbar spinal cord

immune system phenotype

abnormal lymphocyte morphology
- a number of lymphocytes spontaneously form rosettes with autologous erythrocytes in two thirds of the mice

abnormal microglial cell morphology
- increased cell size starting at day 100
- increased granularity starting at day 100
- large vacuoles in microglial cell cytoplasm in the cervical and lumbar spinal cord

abnormal spleen morphology
- diminished follicular architecture with a greater number of follicles at end stage

abnormal splenic cell ratio
- decreased levels of CD45RA+ (naive) T cells
- increased levels of CD45RO+ (memory) T cells

abnormal splenocyte morphology
- increased annexin-V associated apoptosis and necrosis of lymphocytes at pre-symptomatic stage (14 weeks of age)

CNS inflammation
- first in the spinal cord, predominantly in the white matter
- later in the degenerating regions up to the mesencephalon
- lymphocytes and CD11c positive dendritic cells infiltrate the affected CNS regions not before 4 months of age

decreased eosinophil cell number
- decreased number of circulating eosinophils

decreased leukocyte cell number
- Normal - normal neutrophil levels
- reduced WBC numbers

decreased lymphocyte cell number
- lower lymphocyte density

decreased monocyte cell number
- decreased number of circulating monocytes

decreased spleen weight
- reduced spleen weight (45%) at 19 weeks of age

increased basophil cell number
- increased basophil number compared with Tg(SOD1)2Gur mice

microgliosis
- appear simultaneously to the degenerative changes and increase substantially with time
- first noted at 6 weeks
- increase in the number of glial processes abutting onto the neuronal surface and apposed to the presynaptic terminal in 10-week-old and 18-week-old mice
- microglia (CD11b+ CD45lo) population expanded 1.65-fold by day 135 in spinal cords
- some microglial cells occupy areas where formerly occupied by astrocytes or oligodendrocytes in the cervical and lumbar spinal cord

small spleen
- reduced spleen size at end stage (20-22 weeks of age)

mortality/aging

premature death
- Background Sensitivity - decreased survival on SJL/J background (119.2¿9.7 days) in contrast to B6SJL/J background (130.2¿11.2 days)
- female mice survive longer than the male on B6SJL/J (132.8¿12.4 vs. 127.9¿9.5 days) and SJL/J (122.5¿10.9 vs. 115.2¿6.2 days) background
- survival in male mice ranges between 105 days and 127 days
- survival is 157.2 ¿ 2.2 days
- survive from 16-20 weeks

Genotype: Tg(SOD1G93A)1Gur/0
B6SJL-Tg(SOD1G93A)1Gur

behavior/neurological phenotype

abnormal fear-related response
- a stronger amount of freezing and run a shorter distance in comparison with wild-type following the three tone alone presentations on day 3
- impaired extinction of conditioned fear

cellular phenotype

abnormal mitochondrion morphology
- fulminant mitochondrial swelling at 12 weeks of age
- vacuoles in large swollen mitochondria in the cell bodies and proximal dendrites of some motoneurons and in dendrites within in the neuropil at 10 weeks of age

nervous system phenotype

abnormal cholinergic neuron morphology
- decreased density of ChAT-boutons at 16 weeks of age

abnormal dendrite morphology
- reduction in dendrite length and branch nodes on basal dendrites of prelimbic/infralimbic medial prefrontal cortex neurons
- smaller diameter of basal dendrites on branch order one

abnormal dendritic spine morphology
- decreased spine density on basal dendrite segments of branch order four

abnormal motor neuron morphology
- cytoplasmic vacuoles starting at 10 weeks of age
- decreased number of mid-size (20-30 micrometer) motoneurons at 14 weeks of age
- swollen, degenerating axons with segmental enlargements in the ventral horn neuropil and white matter starting at 10 weeks of age
- the average size of motoneurons gradually increases from 10 weeks of age and reaches maximum at 12 weeks
- the residual motoneurons tend to be smaller than that the typical alpha-motoneurons at 16 weeks of age

abnormal neuron morphology
- about 20% lower glycinergic synapses (GlyT2-boutons) densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice
- decreased number of Renshaw cells at 12 and 14 weeks of age
- majority of GlyT2-boutons is lost at 16 weeks of age
- the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice
- the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age

abnormal spinal cord interneuron morphology
- about 20% lower GlyT2-boutons densities on lumbar spinal motoneurons in asymptomatic 8-week-old mice
- decreased density of ChAT-boutons at 16 weeks of age
- decreased number of Renshaw cells at 12 and 14 weeks of age
- majority of GlyT2-boutons is lost at 16 weeks of age
- the density of GlyT2-boutons shows a slight recovery at 10 weeks of age compared with 8-week-old transgenic mice
- the number of GlyT2-boutons is reduced further at 12 and 14 weeks of age

abnormal spinal cord morphology
- cytoplasmic vacuoles in some motoneuron cell bodies and proximal dendrites in lumbar spinal cord at 10 weeks of age
- more apparent cytoplasmic vacuoles in motoneurons and more motoneurons possessed vacuoles at 12 weeks of age

abnormal spinal cord ventral horn morphology
- swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age

abnormal spinal cord white matter morphology
- swollen, degenerating axons with segmental enlargements in the white matter at 10 weeks of age

decreased motor neuron number
- decreased number of motoneurons at 14 weeks of age
- the number of motoneurons is reduced further at 16 weeks of age

motor neuron degeneration
- decreased number of motoneurons at 14 weeks of age
- swollen, degenerating axons with segmental enlargements in the ventral horn neuropil at 10 weeks of age
- the number of motoneurons is reduced further at 16 weeks of age

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6 CD-1 * SJL

nervous system phenotype

decreased neuron number
- decreased number of total neurons within the ventral horns of the lumbar cord at 110 days of age

decreased spinal cord ventral horn cell number
- decreased number of total neurons within the ventral horns of the lumbar cord at 110 days of age

decreased substantia nigra size
- decreased number of total neurons within the substatia nigra at 110 days of age

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6J C57BL/Ola * SJL/J

mortality/aging

premature death
- mice reach end stage by day 131.1¿3.7

nervous system phenotype

decreased sensory neuron number
- lose 53% of total dorsal root axons by day 80

Genotype: Tg(SOD1G93A)1Gur/0
involves: ABH C57BL/6 * SJL

mortality/aging

premature death
- Background Sensitivity - mice on a background containing ABH exhibit shorter life span than mice on a mixed C57BL/6 and SJL background

The following phenotype relates to a compound genotype created using this strain

Genotype: Tg(SOD1G93A)1Gur/0
B6.Cg-Tg(SOD1G93A)1Gur/J

behavior/neurological phenotype

abnormal gait
- exhibited longer stride and stance times as compared to C57BL/6J controls, however swing time difference was not significant

impaired coordination
- mice show impaired performance on the rotarod and in hanging from a grid

long stride length

tremors
- hind limb tremors at 14 weeks of age
- observed hindlimb tremors when suspended by tail at 142.3 +/- 10.6 days (approx. 20 weeks)

immune system phenotype

abnormal spleen morphology
- diminished follicular area by 67% with a greater number (41%) of follicles at end stage

abnormal T cell physiology
- progressively diminished proliferative capacity of T cells with age

decreased spleen weight
- reduced spleen weight (59%) at 22 weeks of age

decreased splenocyte number
- reduced viable spleen cell numbers (70%) at 22 weeks of age

mortality/aging

premature death
- average lifespan is 171.8 days
- die within 10+/-0.6 days of clinical disease onset
- disease onset at 111+/-1.8 days
- increased survival by weekly immunization with Copolymer-1 (9.9%) in female mice
- Background Sensitivity - increased survival on C57BL/6J background (143.6+/-7.5 days) in contrast to B6SJL/J background (130.2+/-11.2 days)
- Background Sensitivity - increased survival on C57BL/6J background (50% survival at 157.1+/-9.3 days) in contrast to B6SJL background (50% survival at 128.9+/-9.1 days)
- maximal life span of 145 days
- mean time from onset of tremors to death is 16.5 +/-9.3 days
- reconstitution with polyclonal-activated Treg and Teff subsets delays loss of motor function and extends survival
- reconstitution with Teff increases latency between disease onset and entry into late stage
- reconstitution with Treg delays neurological symptom onset
- survive from 19-22 weeks

muscle phenotype

abnormal skeletal muscle fiber type ratio
- altered fiber type composition at 80 d with a shift toward a fast fiber type

decreased skeletal muscle fiber diameter
- at 112 d and 123 d compared with wild-type mice
- severe muscle atrophy at 123 d

nervous system phenotype

decreased motor neuron number
- a reduction of 37 and 55% in the number of motor neurons at clinical onset (112 d) and at end-stage disease (123 d), respectively
- progressive reduction in the number of motor neuron from clinical onset to end-stage disease

motor neuron degeneration
- average onset of motor neuron degeneration is 137.4 days

hematopoietic system phenotype

abnormal spleen morphology
- diminished follicular area by 67% with a greater number (41%) of follicles at end stage

abnormal T cell physiology
- progressively diminished proliferative capacity of T cells with age

decreased spleen weight
- reduced spleen weight (59%) at 22 weeks of age

decreased splenocyte number
- reduced viable spleen cell numbers (70%) at 22 weeks of age

Genotype: Tg(SOD1*G93A)1Gur/0
involves: C57BL/6Ico

behavior/neurological phenotype

tremors
- in only 3 of 11 mice
- onset at 185¿6.3 days

growth/size/body region phenotype

weight loss
- declined body weight (12% over 64 days) after 20 weeks of age

mortality/aging

premature death
- decreased survival (208.4¿5.06 vs. >350 days), compared with wild-type mice

Genotype: Tg(SOD1G93A)1Gur/0
B6.Cg-Tg(SOD1G93A)1Gur

behavior/neurological phenotype

decreased grip strength
- deficits in paw grip endurance test (PaGE)
- delayed chronic deficits in female compared to male

decreased vertical activity
- decreased average locomotion velocity and rearing episodes
- deficits in all four open-field performance measures start earlier than the conventional PaGE or rotarod tests
- delayed chronic deficits in average velocity in female compared to male
- motor deficits in rearing episodes beginning at P45
- persist at each subsequent time point up to and beyond the onset of overt clinical symptoms
- significant motor deficits in male and female mice (at P45 and P65 respectively) in average locomotion velocity

hypoactivity
- motor deficits in ambulatory distance and resting time in male mice beginning at P45
- transient or no deficits in ambulatory distance and rest behaviors, respectively in female

impaired coordination
- deficits in rotarod test

tremors
- tremor onset occurred significantly sooner in male (91¿2 days) compared to female (100¿2 days)

growth/size/body region phenotype

decreased body weight
- decreased body weight starting at P65

mortality/aging

premature death
- mean survivals in male and female mice are 153 ¿ 2 and 161¿ 2 days respectively

muscle phenotype

decreased skeletal muscle size
- calf (d/v) and thigh muscle girth are able to predict genotype in pre-symptomatic animals with 80% or greater accuracy (about 40 day prior to tremor onset)
- smaller hindlimb calf girth dorsal/ventral (d/v) starting at P55
- smaller hindlimb calf girth medial/lateral starting at P65
- smaller hindlimb thigh girth medial/lateral starting at P75

References

Gurney ME; Pu H; Chiu AY; Dal Canto MC; Polchow CY; Alexander DD; Caliendo J; Hentati A; Kwon YW; Deng HX; Chen W; Zhai P; Sufit RL; Siddique T. 1994. Motor neuron degeneration in mice that express a human Cu,Zn superoxide dismutase mutation [see comments] [published erratum appears in Science 1995 Jul 14;269(5221):149] Science 264(5166):1772-5PubMed: 8209258 MGI: J:32665
Tu PH; Raju P; Robinson KA; Gurney ME; Trojanowski JQ; Lee VM. 1996. Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions. Proc Natl Acad Sci U S A 93(7):3155-60PubMed: 8610185 MGI: J:76718
Wooley CM; Sher RB; Kale A; Frankel WN; Cox GA; Seburn KL. 2005. Gait analysis detects early changes in transgenic SOD1(G93A) mice. Muscle Nerve 32(1):43-50PubMed: 15880561 MGI: J:115355

Additional References

Andreassen OA; Jenkins BG; Dedeoglu A; Ferrante KL; Bogdanov MB; Kaddurah-Daouk R; Beal MF. 2001. Increases in cortical glutamate concentrations in transgenic amyotrophic lateral sclerosis mice are attenuated by creatine supplementation. J Neurochem 77(2):383-90PubMed: 11299300 MGI: J:68835
Benlefki S; Sanchez-Vicente A; Milla V; Lucas O; Soulard C; Younes R; Gergely C; Bowerman M; Raoul C; Scamps F; Hilaire C. 2020. Expression of ALS-linked SOD1 Mutation in Motoneurons or Myotubes Induces Differential Effects on Neuromuscular Function In vitro. Neuroscience 435:33-43PubMed: 32234507 MGI: J:293165
Chiu AY; Zhai P; Dal Canto MC; Peters TM; Kwon YW; Prattis SM; Gurney ME. 1995. Age-dependent penetrance of disease in a transgenic mouse model of familial amyotrophic lateral sclerosis. Mol Cell Neurosci 6(4):349-62PubMed: 8846004 MGI: J:80625
Fecher C; Trovo L; Muller SA; Snaidero N; Wettmarshausen J; Heink S; Ortiz O; Wagner I; Kuhn R; Hartmann J; Karl RM; Konnerth A; Korn T; Wurst W; Merkler D; Lichtenthaler SF; Perocchi F; Misgeld T. 2019. Cell-type-specific profiling of brain mitochondria reveals functional and molecular diversity. Nat Neurosci 22(10):1731-1742PubMed: 31501572 MGI: J:281635
Guegan C; Vila M; Rosoklija G; Hays AP; Przedborski S. 2001. Recruitment of the mitochondrial-dependent apoptotic pathway in amyotrophic lateral sclerosis. J Neurosci 21(17):6569-76PubMed: 11517246 MGI: J:71177
Kilic E; Weishaupt JH; Kilic U; Rohde G; Yulug B; Peters K; Hermann DM; Bahr M. 2004. The superoxide dismutase1 (sod1) G93A mutation does not promote neuronal injury after focal brain ischemia and optic nerve transection in mice. Neuroscience 128(2):359-64PubMed: 15350647 MGI: J:92468
Lu J; Periz G; Lu YN; Tang Q; Liu Y; Zhang T; Shah Y; Thombre R; Aljumaah R; Li W; Mojsilovic-Petrovic J; Ji Y; Johnson K; Kalb R; Wang J. 2019. L3MBTL1 regulates ALS/FTD-associated proteotoxicity and quality control. Nat Neurosci 22(6):875-886PubMed: 31061493 MGI: J:281537
Murakami T; Ilieva H; Shiote M; Nagata T; Nagano I; Shoji M; Abe K. 2003. Hypoxic induction of vascular endothelial growth factor is selectively impaired in mice carrying the mutant SOD1 gene. Brain Res 989(2):231-7PubMed: 14556945 MGI: J:86204
Pedersen WA; Luo H; Kruman I; Kasarskis E; Mattson MP. 2000. The prostate apoptosis response-4 protein participates in motor neuron degeneration in amyotrophic lateral sclerosis. FASEB J 14(7):913-24PubMed: 10783145 MGI: J:61806
Tadic V; Adam A; Goldhammer N; Lautenschlaeger J; Oberstadt M; Malci A; Le TT; Sengupta S; Stubendorff B; Keiner S; Witte OW; Grosskreutz J. 2019. Investigation of mitochondrial calcium uniporter role in embryonic and adult motor neurons from G93A(hSOD1) mice. Neurobiol Aging 75:209-222PubMed: 30597405 MGI: J:276157
Van Acker ZP; Declerck K; Luyckx E; Vanden Berghe W; Dewilde S. 2019. Non-Methylation-Linked Mechanism of REST-Induced Neuroglobin Expression Impacts Mitochondrial Phenotypes in a Mouse Model of Amyotrophic Lateral Sclerosis. Neuroscience 412:233-247PubMed: 31158439 MGI: J:282904

Additional - Tg(SOD1*G93A)1Gur related

Technical Support

CONTACT TECHNICAL SUPPORT

Genotyping Protocols
- Standard PCR:Tg(SOD1)
- Standard PCR:Tg(SOD)
- Standard PCR:Tg(SOD)
- QPCR:Sod TgN Copy Number
- Genotyping resources and troubleshooting
Dietary Information
- LabDiet® 5K52 formulation (6% fat)
Breeding Considerations

When maintaining the live congenic colony, hemizygous carriers (preferably males) are bred with C57BL/6J inbred mice. Female hemizygotes are poor breeders, and rarely produce more than one litter before the onset of disease. Coat color expected from breeding is black.
- Additional Breeding and Husbandry Support
Mating System
- Inbred x Hemizygote
- C57BL/6J females (000664) x Hemizygot
Citation
When using the B6.SOD1-G93A mouse strain in a publication, please cite the originating article(s) and include JAX stock #004435 in your Materials and Methods section.

Animal Health Reports

Facility Barrier Level Descriptions

AX11 (Maximum)

Pricing & Availability

Available Now

Domestic
International

Live Mouse

Age

Genotype

Price

weeks

Breeder Pair

Sex

Genotype

Price

Female
Male

Cryorecovery - Pricing

Service/Product	Description	Price
	Hemizygous or Non carrier for Tg(SOD1*G93A)1Gur

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Also Known As:B6.SOD1-G93A

Donating Investigator

Genetic overview

Research Applications

Base Price

Detailed Description

Development

Expression Data

Control Suggestions

Additional Information

Selected References

Tg(SOD1*G93A)1Gur

Allele Symbol: Tg(SOD1*G93A)1Gur

Disease Terms

Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.

Research Areas By Phenotype

This mouse can be used to support research in many areas including:

Mammalian Phenotype Terms by Genotype

Genotype: Tg(SOD1*G93A)1Gur/0involves: C57BL/6 * SJL

adipose tissue phenotype

cellular phenotype

growth/size/body region phenotype

homeostasis/metabolism phenotype

behavior/neurological phenotype

mortality/aging

muscle phenotype

nervous system phenotype

Genotype: Tg(SOD1*G93A)1Gur/?involves: C57BL/6 * SJL

behavior/neurological phenotype

growth/size/body region phenotype

mortality/aging

nervous system phenotype

Genotype: Tg(SOD1*G93A)1Gur/0B6SJL-Tg(SOD1*G93A)1Gur/J

behavior/neurological phenotype

muscle phenotype

nervous system phenotype

cardiovascular system phenotype

cellular phenotype

growth/size/body region phenotype

hematopoietic system phenotype

homeostasis/metabolism phenotype

immune system phenotype

mortality/aging

Genotype: Tg(SOD1*G93A)1Gur/0B6SJL-Tg(SOD1*G93A)1Gur

behavior/neurological phenotype

cellular phenotype

nervous system phenotype

Genotype: Tg(SOD1*G93A)1Gur/0involves: C57BL/6 * CD-1 * SJL

nervous system phenotype

Genotype: Tg(SOD1*G93A)1Gur/0involves: C57BL/6J * C57BL/Ola * SJL/J

mortality/aging

nervous system phenotype

Genotype: Tg(SOD1*G93A)1Gur/0involves: ABH * C57BL/6 * SJL

mortality/aging

Genotype: Tg(SOD1*G93A)1Gur/0B6.Cg-Tg(SOD1*G93A)1Gur/J

behavior/neurological phenotype

immune system phenotype

mortality/aging

muscle phenotype

nervous system phenotype

hematopoietic system phenotype

Genotype: Tg(SOD1*G93A)1Gur/0involves: C57BL/6Ico

behavior/neurological phenotype

growth/size/body region phenotype

mortality/aging

Genotype: Tg(SOD1*G93A)1Gur/0B6.Cg-Tg(SOD1*G93A)1Gur

behavior/neurological phenotype

growth/size/body region phenotype

mortality/aging

muscle phenotype

References

Additional References

Additional - Tg(SOD1*G93A)1Gur related

Genotyping Protocols

Dietary Information

Breeding Considerations

Mating System

Citation

Animal Health Reports

Live Mouse

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6 SJL

Genotype: Tg(SOD1G93A)1Gur/?
involves: C57BL/6 SJL

Genotype: Tg(SOD1G93A)1Gur/0
B6SJL-Tg(SOD1G93A)1Gur/J

Genotype: Tg(SOD1G93A)1Gur/0
B6SJL-Tg(SOD1G93A)1Gur

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6 CD-1 * SJL

Genotype: Tg(SOD1G93A)1Gur/0
involves: C57BL/6J C57BL/Ola * SJL/J

Genotype: Tg(SOD1G93A)1Gur/0
involves: ABH C57BL/6 * SJL

Genotype: Tg(SOD1G93A)1Gur/0
B6.Cg-Tg(SOD1G93A)1Gur/J

Genotype: Tg(SOD1*G93A)1Gur/0
involves: C57BL/6Ico

Genotype: Tg(SOD1G93A)1Gur/0
B6.Cg-Tg(SOD1G93A)1Gur