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. Author manuscript; available in PMC: 2015 Oct 13.
Published in final edited form as: Neuron. 2002 Nov 14;36(4):585–596. doi: 10.1016/s0896-6273(02)01045-0

Figure 8. Possible Models to Account for the Correlation between Disease Severity in PMD and DM-20/PLP1 Trafficking Defects.

Figure 8

(A) UPR portrayed as a graded response that is proportional to the level of misfolded protein in the ER; the higher the level, the stronger the UPR. (Aa) Mutations causing ER accumulation of both PLP1 and DM-20 induce a strong UPR. (Ab) Mutations causing accumulation of PLP1 but not DM-20 induce a weak response.

(B) UPR portrayed as an off/on switch upon misfolded protein accumulation. (Ba) PLP1 and DM-20 accumulation induces the UPR. DM-20 is toxic and disrupts cell survival pathways (?). (Bb) Accumulation of only PLP1 in the ER induces the UPR but does not affect cell survival pathways.