Mouse Genome Informatics
cn1
    Isl1tm1(cre)Tmj/Isl1+
Tg(SOD1*G37R)1Dwc/0

involves: 129X1/SvJ * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• overall survival is extended 64 days

nervous system
• in transgenic mice expressing a motorneuron specific Cre, disease onset is delayed 18 days
• progression from onset through early disease is delayed 31 days
• later disease progression is slowed with an average extension of 15 days

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109131


Mouse Genome Informatics
cn2
    Tg(ITGAM-cre)2781Gkl/0
Tg(SOD1*G37R)1Dwc/0

involves: C57BL/6 * CBA
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• expression of Cre in microglia extends the lifespan of mutant SOD1-expressing mice by an average of 99 days compared to non-ITGAM-cre expressing mice

nervous system
• progression of later stage disease is slowed by an average of 75 days in mice expressing both transgenes

Mouse Models of Human Disease
OMIM IDRef(s)
Amyotrophic Lateral Sclerosis 1; ALS1 105400 J:109131


Mouse Genome Informatics
tg3
    Tg(SOD1*G37R)1Dwc/0
involves: C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
mortality/aging
• transgenic mice develop end-stage disease by 8.5-11 months of age

growth/size/body
• mice display weight loss from denervation induced muscle atrophy

nervous system
• mice in end-stage disease display 55% spinal motor neuron death
• mice develop fatal progressive motorneuron disease