Figure 1.

Schematic of the role of the unfolded protein response in myelin structure. Following an endoplasmic reticulum (ER) stress response in Sel1L deficit mice (Suppressor/Enhancer of Lin-12-like), an unfolded protein accumulation inside oligodendrocytes is observed. As a result, the unfolded protein response is activated [as demonstrated by pancreatic ER kinase (PERK) activation and other stress markers] that prevent myelin protein translation. This results in oligodendrocytes degeneration and myelin thinning (Wu et al., 2020). Normal myelin and oligodendrocytes from wild type mice are shown at the bottom.