endoplasmic reticulum stress
A specific inhibitor of extracellular PPIA, MM218, given at symptom onset, rescued motor neurons and extended survival in the SOD1(G93A) mouse model of familial ALS by 11 d. The treatment resulted in the polarization of glia toward a prohealing phenotype associated with reduced NF-kB activation, proinflammatory markers, endoplasmic reticulum stress, and insoluble phosphorylated TDP-43.