Phenotypes associated with this allele

• Allele Symbol: Sod2^tm1Cje
• Allele Name: targeted mutation 1, Charles J Epstein
• Allele ID: MGI:1857344
• Summary: 12 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Sod2^tm1Cje/Sod2^tm1Cje	B6.Cg-Sod2^tm1Cje/Mmmh	MGI:3639893
hm2	Sod2^tm1Cje/Sod2^tm1Cje	D2.Cg-Sod2^tm1Cje	MGI:3639895
hm3	Sod2^tm1Cje/Sod2^tm1Cje	involves: C57BL/6J	MGI:3639891
hm4	Sod2^tm1Cje/Sod2^tm1Cje	involves: C57BL/6J * CD-1	MGI:3639890
hm5	Sod2^tm1Cje/Sod2^tm1Cje	involves: C57BL/6J * DBA/2J	MGI:3639896
ht6	Sod2^tm1Cje/Sod2^+	B6.Cg-Sod2^tm1Cje/Mmmh	MGI:3639892
ht7	Sod2^tm1Cje/Sod2^+	D2.Cg-Sod2^tm1Cje	MGI:3655210
ht8	Sod2^tm1Cje/Sod2^+	involves: C57BL/6J * CD-1	MGI:3639894
ht9	Sod2^tm1Cje/Sod2^+	involves: C57BL/6J * DBA/2J	MGI:3655225
cx10	Sod2^tm1Cje/Sod2^+ Tg(SOD1*G93A)1Gur/0	involves: C57BL/6 * CD-1 * SJL	MGI:4831000
cx11	Sod2^tm1Cje/Sod2^tm1Cje Svtms^C57BL/6J/Svtms^C57BL/6J	involves: C57BL/6J * DBA/2J	MGI:3626356
cx12	Sod2^tm1Cje/Sod2^tm1Cje Svtms^C57BL/6J/Svtms^DBA/2J	involves: C57BL/6J * DBA/2J	MGI:3626357

Genotype
MGI:3639893

hm1

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje
• Genetic Background: B6.Cg-Sod2^tm1Cje/Mmmh

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

perinatal lethality, complete penetrance ( J:73998 )

• mean life span is 0.9 +/- 0.3 days

• at E19 when delivered by cesarean section only 11.6% are homozygous and after natural birth only 8.4% are homozygous

• Background Sensitivity: mean life span is reduced compared to mice on a congenic DBA2/J or hybrid C57BL/6J and DBA2/J background

lethality throughout fetal growth and development, incomplete penetrance ( J:73998 )

• at E15, 4 of 19 fetuses are dead

cardiovascular system

enlarged heart ( J:73998 )

• at E15, moderate to massive enlargement is seen

dilated heart left ventricle ( J:73998 )

• dilated left and right ventricles are seen in severe cases

thin ventricular wall ( J:73998 )

• seen with dilated ventricles in severe cases

dilated heart right ventricle ( J:73998 )

• dilated left and right ventricles are seen in severe cases

dilated cardiomyopathy ( J:73998 )

• at E15, moderate to massive enlargement with the largest hearts having dilated left and right ventricular cavities with thinner muscular walls

• Background Sensitivity: dilated cardiomyopathy is seen on a congenic C57BL/6J background but not in mice on congenic DBA/2J or hybrid C57BL/6J and DBA/2J backgrounds

growth/size/body

enlarged heart ( J:73998 )

• at E15, moderate to massive enlargement is seen

decreased fetal size ( J:73998 )

• at E15, surviving mice are 21% smaller than age-matched wild-type littermates

cellular

oxidative stress ( J:108637 )

• increased cardiomyocyte apoptosis after treatment with t-BuOOH to induce oxidative stress

muscle

dilated cardiomyopathy ( J:73998 )

• at E15, moderate to massive enlargement with the largest hearts having dilated left and right ventricular cavities with thinner muscular walls

• Background Sensitivity: dilated cardiomyopathy is seen on a congenic C57BL/6J background but not in mice on congenic DBA/2J or hybrid C57BL/6J and DBA/2J backgrounds

Genotype
MGI:3639895

hm2

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje
• Genetic Background: D2.Cg-Sod2^tm1Cje

mortality/aging

postnatal lethality, complete penetrance ( J:73998 )

• mean life span is 8.7 +/- 0.3 days

• Background Sensitivity: mean life span is longer than on a congenic C57BL/6J background but shorter than on a hybrid C57BL/6J DBA/2J background

cardiovascular system

increased heart weight ( J:73998 )

• heart weight to body weight ratios are progressively increased with age and show mild hypertrophy but no myocardial inflammation or fibrosis

• Background Sensitivity: no dilated cardiomyopathy is seen unlike mice on a congenic C57BL/6J or CD-1 background

cardiac hypertrophy ( J:73998 )

• mild

homeostasis/metabolism

abnormal blood homeostasis ( J:73998 )

abnormal blood gas level ( J:73998 )

• increase in pO2 and decrease in pCO2 suggest a compensatory increase in respiration as a result of metabolic acidosis

• Background Sensitivity: changes in pO2 and pCO2 are smaller than in mice on a hybrid C57BL/6J DBA/2J background

increased blood urea nitrogen level ( J:73998 )

• Background Sensitivity: seen earlier in congenic mice than in mice on a hybrid C57BL/6J and DBA/2J background

decreased circulating glucose level ( J:73998 )

• Background Sensitivity: seen earlier in congenic mice than in mice on a hybrid C57BL/6J and DBA/2J background

increased circulating ketone body level ( J:73998 )

• Background Sensitivity: higher levels and seen earlier than in homozygotes on a hybrid C57BL/6J and DBA/2J background

abnormal enzyme/coenzyme level ( J:73998 )

• Background Sensitivity: at P5, heart levels of SOD1 activity are increased unlike mice on a hybrid C57BL/6J DBA/2J background where SOD1 activity is also increased in the brain, liver, and lung

increased circulating alkaline phosphatase level ( J:73998 )

• seen in some older mice

increased circulating aspartate transaminase level ( J:73998 )

• seen in some older mice

hypocapnia ( J:73998 )

• a 24% decrease in pCO2 is seen

• Background Sensitivity: decrease is smaller than in mice on a hybrid C57BL/6J and DBA/2J background

hyperoxia ( J:73998 )

• a 20% increase in pO2 is seen

• Background Sensitivity: increase is smaller than in mice on a hybrid C57BL/6J and DBA/2J background

metabolic acidosis ( J:73998 )

• severe metabolic acidosis

• by P5 a rapid decline in HCO3 levels and blood pH is seen

abnormal urine homeostasis ( J:73998 )

• at P5 urinary organic acid levels are elevated

liver/biliary system

hepatic steatosis ( J:73998 )

• Background Sensitivity: large amounts of fat in the liver compared to wild-type or homozygotes on a hybrid C57BL/6J and DBA/2J background

cellular

abnormal mitochondrial physiology ( J:73998 )

• mithochondrial but not cytoplasmice aconitase activity is reduced in the brain, heart, lung, liver, kidney, and skeletal muscle

renal/urinary system

abnormal urine homeostasis ( J:73998 )

• at P5 urinary organic acid levels are elevated

growth/size/body

increased heart weight ( J:73998 )

• heart weight to body weight ratios are progressively increased with age and show mild hypertrophy but no myocardial inflammation or fibrosis

• Background Sensitivity: no dilated cardiomyopathy is seen unlike mice on a congenic C57BL/6J or CD-1 background

cardiac hypertrophy ( J:73998 )

• mild

decreased body weight ( J:73998 )

Genotype
MGI:3639891

hm3

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje
• Genetic Background: involves: C57BL/6J

mortality/aging

postnatal lethality, complete penetrance ( J:45913 )

• die between 3 and 13 days of age

• treatment with Manganese 5, 10 ,15, 20-tetrakis (4-benzoic acid) porpryrin (MnTBAP, a superoxide dismutase mimetic that does not cross the blood-brain barrier) increases survival time to a mean life span of 16.4 days compared to 8.3 days without treatment

cardiovascular system

dilated cardiomyopathy ( J:45913 )

• not detected in mice treated with MnTBAP

nervous system

abnormal brainstem morphology ( J:45913 )

• 9 of 16 mice older than 12 days of age display focal spongiform changes

• vacuoles are most commonly seen in the reticulotegmental nucleus of the pons, the superior and medioventral periolivary nuclei, and in regions of the motor nucleus of cranial nerves V and VII

abnormal cerebral cortex morphology ( J:45913 )

• mice older than 12 days of age display symmetric spongiform changes in large areas of the cerebral cortex, primarily in the mid to lower layers of the grey matter and the immediate subcortical white matter

• vacuoles are mostly present in the neuropil and occasionally in the neuronal perikaryon

abnormal cerebellum morphology ( J:45913 )

• 9 of 16 mice older than 12 days of age display focal spongiform changes

brain vacuoles ( J:45913 )

• no changes in brain morphology are seen in untreated mice before 10 days of age

• many of the vacuoles are surrounded by thin myelin lamellae

abnormal optic nerve morphology ( J:71457 )

• cross-sectional area is smaller; however, the ultrastructure appears normal in 20 to 21 day old MnTBAP treated mice

spongiform encephalopathy ( J:45913 )

• in MnTBAP treated mice

behavior/neurological

impaired righting response ( J:45913 )

• in MnTBAP treated mice

tremors ( J:45913 )

• in MnTBAP treated mice intermittent head tremors develop after P12

ataxia ( J:45913 )

• mice treated with MnTBAP develop progressive ataxia starting in the hindlimbs at about P12 and eventually spreading to the frontlimbs

• older mice display a wide-based gait, sway from side to side, and frequently fall with falls sometimes resulting in multiple rolls

abnormal gait ( J:45913 )

• at about P12 MnTBAP treated mice develop ataxia in the hindlimbs with alternating extensor dystonic-like posturing of the hindlimbs and pivoting on the extended limb

vision/eye

abnormal optic nerve morphology ( J:71457 )

• cross-sectional area is smaller; however, the ultrastructure appears normal in 20 to 21 day old MnTBAP treated mice

abnormal retina morphology ( J:71457 )

• in 20 to 21 day old MnTBAP treated mice total retinal thickness, peripheral retinal thickness, and thickness of the combined nerve fiber, ganglion cell, and inner plexiform layers are reduced

• unlike wild-type mice total retinal thickness does not increase between 9-10 and 20-21 days of age

abnormal retina pigment epithelium morphology ( J:71457 )

• abnormal mitochondria are more common at 16 and 20-21 days of age in MnTBAP treated mice compared to controls

• mitochondrial abnormalities include foci of swelling, matrix pallor, and disorganization of the cristae

thin retina inner nuclear layer ( J:71457 )

• thinner at 20-21 days of age in MnTBAP treated mice

abnormal retina photoreceptor layer morphology ( J:71457 )

• the central and overall photoreceptor layer are thinner at 9-10 and 20-21 days of age, respectively, in MnTBAP treated mice

liver/biliary system

hepatic steatosis ( J:45913 )

• lipid accumulation is decreased with MnTBAP treatment

growth/size/body

postnatal growth retardation ( J:45913 )

• weight gain is improved with MnTBAP treatment

muscle

dilated cardiomyopathy ( J:45913 )

• not detected in mice treated with MnTBAP

pigmentation

abnormal retina pigment epithelium morphology ( J:71457 )

• abnormal mitochondria are more common at 16 and 20-21 days of age in MnTBAP treated mice compared to controls

• mitochondrial abnormalities include foci of swelling, matrix pallor, and disorganization of the cristae

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
amyotrophic lateral sclerosis type 1		DOID:0060193	OMIM:105400	J:45913
Canavan disease		DOID:3613	OMIM:271900	J:45913
Leigh disease		DOID:3652	OMIM:256000	J:45913

Genotype
MGI:3639890

hm4

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje
• Genetic Background: involves: C57BL/6J * CD-1

mortality/aging

postnatal lethality, complete penetrance ( J:29899 )

• by 4 - 5 days after birth, 37.5% are dead and almost all die by 10 days of age

cellular

abnormal cell physiology ( J:52592 )

• an increase in oxidative DNA lesions is seen in cells from the heart and brain, but not in liver cells

abnormal aerobic respiration ( J:52592 )

• at 4 to 6 days of age, succinate dehydrogenase (complex II + III) activity is reduced to 35% and 24% of control in mitochondria from skeletal muscle and heart, respectively

• at 4 to 6 days of age, complex I and citrate synthase activity are reduced to 59% and 32% of control in heart

• liver mitochondria show a 36% decrease in 3-hydroxy-3-methylglutaryl-CoA lyase activity

abnormal tricarboxylic acid cycle ( J:29899 , J:52592 )

• aconitase activity is reduced by about 43%, 37%, and 22% in the heart, liver, and brain, respectively; however at P4 - 5 mitochondrial morphology appears normal (J:29899)

• at 9 to 10 days of age aconitase activity is reduced by 89% and 67-76% in the heart and various regions of the brain, respectively (J:52592)

cardiovascular system

cardiac hypertrophy ( J:29899 )

• ventricular

cardiac fibrosis ( J:29899 )

• endocardial fibrosis

dilated cardiomyopathy ( J:29899 )

• enlarged hearts with a dilated left ventricular, reduced left ventricular wall thickness, hypertrophy and endocardial fibrosis but no signs of heart failure are seen

liver/biliary system

increased liver weight ( J:29899 )

• weight is increased relative to body weight

hepatic steatosis ( J:29899 )

abnormal liver physiology ( J:29899 )

• slightly decreased glutamate oxaloacetate transaminase levels but normal glutamate pyruvate transaminase and bilirubin levels

behavior/neurological

fatigue ( J:29899 )

• fatigue rapidly after any exertion

homeostasis/metabolism

increased circulating ketone body level ( J:29899 )

decreased circulating lactate level ( J:29899 )

• lower serum lactate concentration

decreased body surface temperature ( J:29899 )

• neonates have surface temperatures that are 2.6 degrees C lower than controls

calcinosis ( J:29899 )

• calcification of the submucosal regions of the small intestine

metabolic acidosis ( J:29899 )

• compensated metabolic acidosis

organic aciduria ( J:52592 )

• at 9, 10, 14 and 15 days of age increased concentrations of organic acids are found in the urine

hematopoietic system

decreased hematocrit ( J:29899 )

• seen in neonates

muscle

dilated cardiomyopathy ( J:29899 )

• enlarged hearts with a dilated left ventricular, reduced left ventricular wall thickness, hypertrophy and endocardial fibrosis but no signs of heart failure are seen

abnormal skeletal muscle morphology ( J:29899 )

• lipid deposits

hypotonia ( J:29899 )

• seen in neonates

growth/size/body

cardiac hypertrophy ( J:29899 )

• ventricular

postnatal growth retardation ( J:29899 )

• mice that survive to 4 - 5 days of age are severely growth retarded

increased liver weight ( J:29899 )

• weight is increased relative to body weight

renal/urinary system

organic aciduria ( J:52592 )

• at 9, 10, 14 and 15 days of age increased concentrations of organic acids are found in the urine

integument

pallor ( J:29899 )

Genotype
MGI:3639896

hm5

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje
• Genetic Background: involves: C57BL/6J * DBA/2J

mortality/aging

postnatal lethality, complete penetrance ( J:73998 )

• mean life span is 15.7 +/- 0.5 days

• Background Sensitivity: mean life span is longer than on a congenic C57BL/6J or DBA/2J background

cardiovascular system

increased heart weight ( J:73998 )

• heart weight to body weight ratios are progressively increased with age and show mild hypertrophy but no myocardial inflammation or fibrosis

• Background Sensitivity: no dilated cardiomyopathy is seen unlike mice on a congenic C57BL/6J or CD-1 background

cardiac hypertrophy ( J:73998 )

• mild

homeostasis/metabolism

abnormal blood homeostasis ( J:73998 )

abnormal blood pH regulation ( J:73998 )

• Background Sensitivity: a steady mild decline in HCO3 levels is seen resulting in maintenance of blood pH in the normal range through P15, unlike the rapid decline seen in mice on a congenic DBA2/J background

increased blood urea nitrogen level ( J:73998 )

• Background Sensitivity: seen later in hybrid mice than in mice on a congenic DBA/2J background

decreased circulating glucose level ( J:73998 )

• Background Sensitivity: seen later in hybrid mice than in mice on a congenic DBA/2J background

increased circulating ketone body level ( J:73998 )

• Background Sensitivity: lower than in homozygotes on a congenic DBA/2J background

abnormal enzyme/coenzyme level ( J:73998 )

• Background Sensitivity: at P5, brain, heart, lung, and liver levels of SOD1 activity are increased unlike mice on a congenic DBA/2J background where SOD1 activity is increased only in the heart

hypocapnia ( J:73998 )

• a 40% decrease in pCO2 is seen

• Background Sensitivity: decrease is larger than in mice on a congenic DBA/2J background

hyperoxia ( J:73998 )

• a 55% increase in pO2 is seen

• Background Sensitivity: increase is larger than in mice on a congenic DBA/2J background

acidosis ( J:73998 )

• Background Sensitivity: milder than in mice on a congenic DBA/2J background

abnormal urine homeostasis ( J:73998 )

• at P10 and P15 urinary organic acid levels are elevated but no evidence of lactic acidosis is seen at P15

liver/biliary system

liver/biliary system phenotype ( J:73998 )

N • Background Sensitivity: do not accumulate large amounts of lipid in the liver unlike mice on a congenic DBA/2J background

cellular

abnormal tricarboxylic acid cycle ( J:73998 , J:98007 )

• mitochondrial but not cytoplasmic aconitase activity is reduced in the brain, heart, lung, liver, kidney, and skeletal muscle (J:73998)

• mitochondrial aconitase activities are reduced by 72%, 61%,60%, and 62% in the cortex, thalamus, hippocampus, and brainstem, respectively (J:98007)

behavior/neurological

tremors ( J:98007 )

• visible tremors at P11

ataxia ( J:73998 , J:98007 )

• progressive ataxia with age (J:73998)

• at P11 unsteady movement are seen progressing rapidly to truncal instability (J:98007)

abnormal gait ( J:98007 )

• tendency to push or walk backward

seizures ( J:73998 , J:98007 )

• frequent seizures are seen in older mice (J:73998)

• by P14, frequent spontaneous seizures are seen (J:98007)

renal/urinary system

abnormal urine homeostasis ( J:73998 )

• at P10 and P15 urinary organic acid levels are elevated but no evidence of lactic acidosis is seen at P15

nervous system

seizures ( J:73998 , J:98007 )

• frequent seizures are seen in older mice (J:73998)

• by P14, frequent spontaneous seizures are seen (J:98007)

abnormal brain morphology ( J:98007 )

abnormal brainstem morphology ( J:98007 )

• vacuolar degeneration is seen in discrete regions

abnormal trigeminal V mesencephalic nucleus morphology ( J:98007 )

• degenerative changes are detected by P11-13

abnormal trigeminal motor nucleus morphology ( J:98007 )

• degenerative changes are detected by P11-13

abnormal forebrain morphology ( J:98007 )

abnormal thalamus morphology ( J:98007 )

• vacuolar degeneration is seen in discrete regions

abnormal hippocampus morphology ( J:98007 )

• vacuolar degeneration is seen in discrete regions

abnormal frontal lobe morphology ( J:98007 )

• vacuolar degeneration is seen in deep layers of the motor cortex by P11-13

neuron degeneration ( J:98007 )

• in regions of vacuolar degeneration, enlarged and degenerating mitochondria are found in the neurons and axons and the thickness of the myelin sheaths are reduced

• degeneration is first seen around P11-13 and becomes more extensive by P15

axon degeneration ( J:98007 )

• in regions of vacuolar degeneration, enlarged and degenerating mitochondria are found in the neurons and axons and the thickness of the myelin sheaths are reduced

muscle

abnormal skeletal muscle fiber morphology ( J:98007 )

• proliferation of mitochondria located in clusters near the sarcolemmal membrane

decreased skeletal muscle fiber size ( J:98007 )

growth/size/body

increased heart weight ( J:73998 )

• heart weight to body weight ratios are progressively increased with age and show mild hypertrophy but no myocardial inflammation or fibrosis

• Background Sensitivity: no dilated cardiomyopathy is seen unlike mice on a congenic C57BL/6J or CD-1 background

cardiac hypertrophy ( J:73998 )

• mild

decreased body weight ( J:73998 )

Genotype
MGI:3639892

ht6

• Allelic Composition: Sod2^tm1Cje/Sod2⁺
• Genetic Background: B6.Cg-Sod2^tm1Cje/Mmmh

mortality/aging

mortality/aging ( J:87514 )

N • no difference in life span or biochemical and physiological indices of aging are detected

neoplasm

increased tumor incidence ( J:87514 )

• more than 80% have neoplastic lesions by 26-28 months of age compared to 41% of wild-type mice; however the types and severity of lesions are similar to those in wild-type mice

• the incidence of mice with multiple tumor types is increased to 66.6% compared to 18.5% in wild-type mice

increased lymphoma incidence ( J:87514 )

• incidence is increased to 61% compared to 22% in wild-type mice

cellular

abnormal mitochondrial physiology ( J:108637 )

• aconitase and NADH-oxidoreductase (with CoQ as a substrate) activities are reduced by 35% and 45%, respectively, in hearts; however, mitochondrial fumerase and cytosolic aconitase and glutamine synthetase activities are not reduced

• the speed of induction of permeability transition by calcium is significantly increased in heart mitochondria

abnormal respiratory electron transport chain ( J:108637 )

• the respiratory control ratio for complex I and state 3 respiration (with glutamate and malate as substrates) are reduced by 37%; however, state 4 respiration is similar to controls

oxidative stress ( J:87514 , J:108637 )

• significantly higher levels of oxidative DNA damage (measured as 8oxodG) are detected in mitochondrial and nuclear DNA at all ages examined (J:87514)

• treatment with 50 mg paraquat / kg resulted in loss of 30% of heterozygotes within 4 days, unlike wild-type mice which all survived (J:87514)

• increased cardiomyocyte apoptosis after treatment with t-BuOOH to induce oxidative stress compared to controls but less than in homozygous cells (J:108637)

Genotype
MGI:3655210

ht7

• Allelic Composition: Sod2^tm1Cje/Sod2⁺
• Genetic Background: D2.Cg-Sod2^tm1Cje

cellular

abnormal mitochondrial physiology ( J:73998 )

• at P5 mithochondrial but not cytoplasmice aconitase activity is reduced in the brain, heart, lung, liver, kidney, and skeletal muscle

• this reduction is not as severe as in homozygotes

Genotype
MGI:3639894

ht8

• Allelic Composition: Sod2^tm1Cje/Sod2⁺
• Genetic Background: involves: C57BL/6J * CD-1

cellular

increased hepatocyte apoptosis ( J:67874 )

• increased 3-fold at 20 - 25 months of age

abnormal mitochondrial physiology ( J:67874 )

• liver mitochondria are more prone to permeability transition following calcium addition

• liver mitochondrial membrane electrochemical potential is lower than in wild-type

• liver mitochondrial oxidative phosphorylation complexes I, II, II + III, and IV and citrate synthase are elevated 25% - 75% in 20 to 25 month old mice

abnormal respiratory electron transport chain ( J:67874 )

• state III respiratory rate is reduced in liver cells at 5 and 10 - 14 months of age, but by 20 - 25 months control rates decrease so that they are similar to homozygotes

• state IV respiration rate is increased in 10 - 14 and 20 - 25 month old mice but not in young (5 month old) mice

• state III respiratory control ratios are reduced in young and middle aged mice but closer to normal in old mice

oxidative stress ( J:67874 )

• at 10 - 14 months of age, liver mitochondria contain twice as much lipid hydroperoxides as in controls, but lipid peroxide levels decrease in old homozygotes

liver/biliary system

increased hepatocyte apoptosis ( J:67874 )

• increased 3-fold at 20 - 25 months of age

Genotype
MGI:3655225

ht9

• Allelic Composition: Sod2^tm1Cje/Sod2⁺
• Genetic Background: involves: C57BL/6J * DBA/2J

cellular

abnormal tricarboxylic acid cycle ( J:73998 )

• at P5 mitochondrial but not cytoplasmic aconitase activity is reduced in the brain, heart, lung, liver, kidney, and skeletal muscle

• this reduction is not as severe as in homozygotes

Genotype
MGI:4831000

cx10

• Allelic Composition: Sod2^tm1Cje/Sod2⁺; Tg(SOD1*G93A)1Gur/0
• Genetic Background: involves: C57BL/6 * CD-1 * SJL

mortality/aging

premature death ( J:62381 )

• accelerated disease progression

• shorter lifespans than Tg(SOD1*G93A)1Gur mice

• mean decrease in survival is 11 days

behavior/neurological

impaired coordination ( J:62381 )

• worse motor performance in rotorod test than Tg(SOD1*G93A)1Gur mice starting from 106 days of age

nervous system

decreased substantia nigra size ( J:62381 )

• decreased number of total neurons within the substatia nigra at 110 days of age

abnormal motor neuron morphology ( J:62381 )

• microvesiculation within large ventral horn motor neurons at 90 days of age

decreased neuron number ( J:62381 )

• accelerated disease progression

• decreased number of total neurons within the ventral horns of the lumbar cord at 90 days of age

• greater neuronal loss than that in Tg(SOD1*G93A)1Gur mice at 110 days of age

decreased spinal cord ventral horn cell number ( J:62381 )

• decreased number of total neurons within the ventral horns of the lumbar cord starting at 90 days of age

• greater neuronal loss than that in Tg(SOD1*G93A)1Gur mice at 110 days of age

decreased spinal cord size ( J:62381 )

• atrophy of the lumbar spinal cord starting at 90 days of age

Genotype
MGI:3626356

cx11

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje; Svtms^C57BL/6J/Svtms^C57BL/6J
• Genetic Background: involves: C57BL/6J * DBA/2J

mortality/aging

postnatal lethality, complete penetrance ( J:108213 )

• increased postnatal lethality

• shorter survival time (less than 6 days)

Genotype
MGI:3626357

cx12

• Allelic Composition: Sod2^tm1Cje/Sod2^tm1Cje; Svtms^C57BL/6J/Svtms^DBA/2J
• Genetic Background: involves: C57BL/6J * DBA/2J

mortality/aging

mortality/aging ( J:108213 )

N • increased survival time (>6 days)

N • decreased postnatal lethality