Statements (50)
| Predicate | Object |
|---|---|
| gptkbp:instanceOf |
animal model of ALS
transgenic mouse model |
| gptkbp:backgroundStrain |
gptkb:C57BL/6
B6SJL |
| gptkbp:chromosomeIntegration |
random
|
| gptkbp:copyNumber |
high
|
| gptkbp:depot |
gptkb:The_Jackson_Laboratory
|
| gptkbp:developedBy |
gptkb:Gurney_et_al.
|
| gptkbp:expressedIn |
human SOD1 gene with G93A mutation
|
| gptkbp:firstDescribed |
1994
|
| gptkbp:hasPhenotype |
paralysis
muscle weakness motor neuron degeneration reduced lifespan |
| https://www.w3.org/2000/01/rdf-schema#label |
SOD1-G93A transgenic mouse
|
| gptkbp:inheritance |
autosomal dominant
|
| gptkbp:JAXStockNumber |
002726
|
| gptkbp:lifespan |
~120 days
|
| gptkbp:motorDeficits |
progressive
|
| gptkbp:mutationAminoAcidChange |
glycine to alanine
|
| gptkbp:mutationAssociatedWith |
missense
G93A in SOD1 gene |
| gptkbp:mutationPosition |
codon 93
|
| gptkbp:onsetOfSymptoms |
early adulthood
|
| gptkbp:pathology |
muscle atrophy
astrocytosis axonal degeneration inclusion bodies microgliosis |
| gptkbp:relatedTo |
gptkb:familial_ALS
gptkb:sporadic_ALS |
| gptkbp:sexDifference |
minimal
|
| gptkbp:species |
gptkb:Mus_musculus
|
| gptkbp:transgene |
gptkb:human_SOD1
|
| gptkbp:usedBy |
ALS researchers
|
| gptkbp:usedFor |
amyotrophic lateral sclerosis research
preclinical drug testing studying axonal transport studying cell death pathways studying excitotoxicity studying glial cell function studying mitochondrial dysfunction studying motor neuron disease studying neurodegeneration studying neuroinflammation studying oxidative stress studying protein aggregation testing gene therapies |
| gptkbp:bfsParent |
gptkb:SOD1
|
| gptkbp:bfsLayer |
5
|