Mutations in the SOD1 gene cause motor neurons to die in amyotrophic lateral sclerosis, also known as Lou Gehrig's disease. Hidenori Ichijo of the University of Tokyo and his co-workers have pinned down why.
The key lies in the system of intracellular membranes called the endoplasmic reticulum (ER). Mutations in SOD1 seem to affect the system that degrades worn-out pieces of ER, and a surfeit of ER containing misfolded proteins activates a genetic programme that kills the cell.
Ichijo's team found that they could mitigate motor-neuron death and extend the lifespan of SOD1-mutant mice by deleting a gene (ASK1) that turns on the cell-death programme.
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Molecular biology: Sod it. Nature 453, 960 (2008). https://doi.org/10.1038/453960c
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DOI: https://doi.org/10.1038/453960c
