General Information:

Id: 777
Diseases: Amyotrophic lateral sclerosis
Mus musculus
SOD1G93A transgenic mouse, transfected with human SOD1G93A
BTO:0001103 skeletal muscle
article
Reference: Muller FL et al.(2007) Denervation-induced skeletal muscle atrophy is associated with increased mitochondrial ROS production Am. J. Physiol. Regul. Integr. Comp. Physiol. 293 [PMID: 17584954]

Interaction Information:

Comment ROS production is increased in muscle mitochondria in mouse models of ALS and is correlated with the extent of muscle atrophy. The decrease of muscle mass began as early as 60-70 days in the G93A mutants, even before visible symptoms occurred at the age of onset. Late-stage G93A mice exhibited decreases in hindlimb mass approaching 80%.
Formal Description
Interaction-ID: 4365

gene/protein mutant

SOD1-p.G93A

increases_activity of

process

muscle atrophy

in skeletal muscle
Comment ROS production is increased in muscle mitochondria in mouse models of ALS and is correlated with the extent of muscle atrophy. State 1 ROS release during the disease course in G93A mice was increased almost 10-fold above levels in the mitochondria from wild-type mice, much higher than in the Sod1(-/-) mice or in old wild- type mice, which had relatively less muscle atrophy.
Formal Description
Interaction-ID: 4367

gene/protein mutant

SOD1-p.G93A

increases_quantity of

drug/chemical compound

Reactive oxygen species

in skeletal muscle mitochondria