Mouse Genome Informatics
cx1
    Tg(SOD1)2Gur/0
Tg(SOD1*G85R)#Roos/0

involves: C57BL/6 * C57BL/6J * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• mean survival is 185 days

behavior/neurological
• progressive paralysis
• earlier onset (at about 131 days of age) of disease than single Tg(SOD1*G85R)#Roos mutants (at around 307 days of age), however disease duration is similar

growth/size/body
• weight loss begins at 131 days of age

nervous system
• in the anterior horn of the spinal cord at 150 days of age
• SOD1-immunoreactive aggregates (of G85R mutant and wild-type SOD1 heterodimers, wild-type SOD1 homodimers and G85R mutant homodimers) are seen in motor neuron cells in the anterior horn at 150 days of age
• loss of motor neurons at 150 days of age and loss of motor neuron connections with muscle in the lumbar spinal cord anterior horn

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:147156


Mouse Genome Informatics
cx2
    Tg(SOD1)2Gur/0
Tg(Thy1-SOD1*G93A)T3Hgrd/0

involves: C57BL/6 * CBA * FVB * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• ubiquinated SOD1 aggregates are seen at >45 weeks
• muscle denervation is seen at end-stage of disease (<80 weeks)
• motor neuron loss characterizes end-stage of disease (<80 weeks)
• ubiquinated dendritic SOD1 aggregates develop; these are present as early as 15-20 weeks

behavior/neurological
• most mice show total paralysis of at least one hind limb; at end-stage, >80% of mice display fully immobilized hindlimbs

muscle
• develops at about 1 year of age
• reach end-stage of disease before 80 weeks
• 60% of animals show hindlimb onset of disease while subset displays forelimb onset with short disease duration


Mouse Genome Informatics
cx3
    Tg(SOD1)2Gur/0
Tg(SOD1*G93A)1Gur/0

involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• lifespan is on average 117.7 days

nervous system
• mutants exhibit exacerbated amyotrophic lateral sclerosis-like phenotypes compared to single Tg(SOD1*G93A)1Gur mice, with onset of disease on average at 76.8 days of age

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458


Mouse Genome Informatics
cx4
    Tg(SOD1)2Gur/0
Tg(SOD1*A4V)A1073Gur/0

involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• lifespan is on average 318.5 days

nervous system
• mutants exhibit protein aggregates in spinal cords
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 227.4 days

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458


Mouse Genome Informatics
cx5
    Tg(SOD1)2Gur/0
Tg(SOD1*L126Z)#Deng/0

involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• lifespan is on average 201.4 days

nervous system
• mutants exhibit protein aggregates, composed of both mutant and wild-type SOD1, in spinal cords
• mutants develop an amyotrophic lateral sclerosis-like phenotype, with an average onset of disease at 178.3 days

cellular
• mutants exhibit protein aggregation in spinal cord mitochondria, resulting in severely damaged cristae

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109458


Mouse Genome Informatics
cx6
    Tg(Prnp-CCS)17Jlel/?
Tg(SOD1)2Gur/?

involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
behavior/neurological
N
• no neurological disease is observed (J:120361)


Mouse Genome Informatics
tg7
    Tg(SOD1)2Gur/0
involves: C57BL/6 * CBA * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
nervous system
• ubiquinated SOD1 aggregates accumulate in oligodendrocytes of spinal cords of aged mice (>70 weeks) but a much lower density than in double transgenic T3/SOD1 animals


Mouse Genome Informatics
tg8
    Tg(SOD1)2Gur/0
involves: C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype

Amyotrophic lateral sclerosis-like lesions in the spinal cord of Tg(SOD1)2Gur/0 and Tg(SOD1-G93A)1Gur/0 mice

nervous system
• mild swelling of axons traveling toward the anterior roots at 232 days of age (J:78629)
• numerous small vacuoles in the axoplasm of some swollen axons (J:78629)
• never show any clinical signs of disease at up to 300 days of age (J:78630)
• develop neurofilament-rich spheroids in the spinal cords at much later time (132 days of age) points than Tg(SOD1-G93A)1Gur mutants, however do not appear to develop motor neuron disease