Quiescin-sulfhydryl oxidase inhibits prion formation in vitro

Yi An Zhan, Romany Abskharon, Yu Li, Jue Yuan, Liang Zeng, Johnny Dang, Manuel Camacho Martinez, Zerui Wang, Jacqueline Mikol, Sylvain Lehmann, Shizhong Bu, Jan Steyaert, Li Cui, Robert B. Petersen, Qingzhong Kong, Gong Xiang Wang, Alexandre Wohlkonig, Wen Quan Zou

Research output: Contribution to journalArticlepeer-review

2 Scopus citations


Prions are infectious proteins that cause a group of fatal transmissible diseases in animals and humans. The scrapie isoform (PrPSc) of the cellular prion protein (PrPC) is the only known component of the prion. Several lines of evidence have suggested that the formation and molecular features of PrPSc are associated with an abnormal unfolding/refolding process. Quiescin-sulfhydryl oxidase (QSOX) plays a role in protein folding by introducing disulfides into unfolded reduced proteins. Here we report that QSOX inhibits human prion propagation in protein misfolding cyclic amplification reactions and murine prion propagation in scrapieinfected neuroblastoma cells. Moreover, QSOX preferentially binds PrPSc from prion-infected human or animal brains, but not PrPC from uninfected brains. Surface plasmon resonance of the recombinant mouse PrP (moPrP) demonstrates that the affinity of QSOX for monomer is significantly lower than that for octamer (312 nM vs 1.7 nM). QSOX exhibits much lower affinity for N-terminally truncated moPrP (PrP89-230) than for the full-length moPrP (PrP23-231) (312 nM vs 2 nM), suggesting that the N-terminal region of PrP is critical for the interaction of PrP with QSOX. Our study indicates that QSOX may play a role in prion formation, which may open new therapeutic avenues for treating prion diseases.

Original languageEnglish
Pages (from-to)3419-3429
Number of pages11
Issue number12
StatePublished - 2016


  • Prions
  • Protein misfolding cyclic amplification
  • Quiescin sulfhydryl oxidase (QSOX)
  • Recombinant prion protein
  • Scrapie-infected mouse neuroblastoma cells (ScN2a)


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