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. 2021 Nov 8;18(4):2236–2268. doi: 10.1007/s13311-021-01145-z

Table 3.

Treatment targets in MPZ-associated neuropathy

Target Mutation/model Intervention Reference
UPR pathway S63del CHOP ablation Sharifov (2013)
GADD34 inhibition D’Antonio (2013)
Salubrinol Boyce (2005), D’Antonio (2013)
Sephin1 Das (2015)
R98C Curcumin derivatives Patzko 2012
ERAD pathway S63del N-Acetyl-D-glucosamine (GlcNAc) Volpi (2019)
Axonal neuregulin type III S63del Overexpression of Nrg1TIII Scapin (2019)
Suppression of Nrg1TIII inhibitor (TACE/ADAM17) Scapin (2019)
NaV1.8 sodium channels MPZ-deficient mice NaV1.8 blocker Rosberg (2016)

UPR unfolded protein response, CHOP CCAAT-enhancer-binding protein homologous protein, GADD34 growth arrest and DNA damage-inducible protein, SERCA sarcoplasmic/endoplasmic reticulum Ca2 + -ATPase, ERAD endoplasmic-reticulum-associated protein degradation, Nrg1TIII axonal neuregulin 1 type III, TACE tumor necrosis factor-α-converting enzyme, ADAM17 A disintegrin and metalloprotease 17, MPZ myelin protein zero