Mouse Models
SPG7
Publication
Spg7−/−Afg3l2Emv66/+ mice
Identification
- Causal gene(s)
- SPG7
- Repeat size or mutation
- Spg7 homozygous knockout and heterozygous Afg3l2 mice
- Animal model
- Mouse
Model Details
- Mouse strain / background
- C57BL6-129Sv x FVB
- Type of model
- knockout
Phenotype
- Progression
- progressive
- Hallmark features
- Loss of balance, tremor, ataxia, axonopathy, cerebellar degeneration, loss of Purkinje cells and parallel fibers, reactive astrogliosis, mitochondrial abnormalities
Source & Publications
- Originating lab / institution
- Istituto Neurologico C. Besta
- Links to publications or public resources
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