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Transgene Detail
Symbol: Tg(SOD1*H46R*H48Q)58Dbo
Name: transgene insertion 58, David Borschelt
MGI ID: MGI:3695884
Transgene: Tg(SOD1*H46R*H48Q)58Dbo  Location: unknown  
Strain of Origin:  (C3H/HeJ x C57BL/6J)F2
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
Tg(SOD1*H46R*H48Q)58Dbo expresses 1 gene
Mutation detailsA 12kb genomic fragment containing the entire human SOD1 gene was mutated so that the histidine residue at position 46 was replaced by arginine and the histidine at position 48 was replaced by glutamine in the encoded protein. This line is the representative line of 10 that developed motor neuron disease before 1 year of age. Lines 73, 67, and 58 show identical pathological changes. (J:119631)
View phenotypes and curated references for all genotypes (concatenated display).
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Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 0 strains available      Cell Lines: 0 lines available
Original:  J:119631 Wang J, et al., Fibrillar inclusions and motor neuron degeneration in transgenic mice expressing superoxide dismutase 1 with a disrupted copper-binding site. Neurobiol Dis. 2002 Jul;10(2):128-38
All:  8 reference(s)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB), Gene Ontology (GO)
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MGI 6.16
The Jackson Laboratory