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SOD1(G93A)

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Aliases

G93A, mutant SOD1(G93A)

Evidence Snippets

Here, we examined mitochondrial dynamics in live skeletal muscle of an ALS mouse model (G93A) harboring a superoxide dismutase mutation (SOD1(G93A)).
Evidence 1Source 1DOIPubMedprovenance

Supporting Sources

Linked Claims

Claim 1causal implicationsupports2013Source 1DOIPubMed

Overexpression of mutant SOD1(G93A) in skeletal muscle of normal mice induces abnormalities in mitochondrial dynamics, supporting that the SOD1 mutation can drive ALS-like muscle pathology without motor neuron degeneration.

Quoted textsource-backed
similar abnormalities in mitochondrial dynamics were induced by overexpression of mutant SOD1(G93A) in skeletal muscle of normal mice, indicating the SOD1 mutation drives ALS-like muscle pathology in the absence of motor neuron degeneration
Claim 2causal modelsupports2013Source 1DOIPubMed

Accumulation of mutant SOD1(G93A) inside mitochondria, mitochondrial membrane depolarization, and abnormal mitochondrial dynamics are causally linked and cause intrinsic muscle pathology early in ALS.

Quoted textsource-backed
Our results suggest that accumulation of mutant SOD1(G93A) inside mitochondria, depolarization of mitochondrial membrane potential and abnormal mitochondrial dynamics are causally linked and cause intrinsic muscle pathology, which occurs early in the course of ALS
Claim 3intervention effectsupports2013Source 1DOIPubMed

Mdivi-1 reverses the effect of SOD1(G93A) on mitochondrial dynamics, supporting that SOD1(G93A) promotes mitochondrial fission.

Quoted textsource-backed
A specific mitochondrial fission inhibitor (Mdivi-1) reversed the SOD1(G93A) action on mitochondrial dynamics, indicating SOD1(G93A) likely promotes mitochondrial fission process.
Claim 4mechanistic observationsupports2013Source 1DOIPubMed

Mutant SOD1(G93A) forms aggregates inside muscle mitochondria and is associated with mitochondrial network fragmentation and mitochondrial depolarization.

Quoted textsource-backed
Mutant SOD1(G93A) forms aggregates inside muscle mitochondria and leads to fragmentation of the mitochondrial network as well as mitochondrial depolarization.
Claim 5observed effectsupports2013Source 1DOIPubMed

Abnormal mitochondrial dynamics are present in skeletal muscle of young SOD1(G93A) mice before disease onset.

Quoted textsource-backed
we discovered abnormal mitochondrial dynamics in skeletal muscle of young G93A mice before disease onset