Improved mitochondrial calcium buffering did not extend survival in ALS mice
Original source
Enhancing mitochondrial calcium buffering capacity reduces aggregation of misfolded SOD1 and motor neuron cell death without extending survival in mouse models of inherited amyotrophic lateral sclerosis. (opens in a new tab)Compass summarised this from the study's abstract.
Study details
- Studied in
- Human, Mouse
Related topics
Increasing mitochondria’s ability to store calcium reduced misfolded SOD1 aggregates, motor neuron death and mitochondrial damage in three mouse models of inherited ALS. However, muscle denervation, motor axon loss, disease progression and survival were unchanged.
Why this matters
The findings suggest that these mitochondrial changes and motor neuron loss were not the main drivers of fatal paralysis in these mouse models. This was animal research and does not change treatment for people with ALS.
Limitations and context
The study used genetically modified mice with inherited SOD1 mutations, not people with ALS. It tested removal of cyclophilin D, a specific genetic intervention, and was published in 2013. The improvements in cellular and motor-neuron measures did not improve survival, so further research would be needed to determine whether the findings apply to other ALS types or treatments.