title: [Mutant D178N prion protein converts spontaneously in RT-QuIC assay].
abstract: OBJECTIVE
  To study the conversion of mutant D178N prion protein in RT-QuIC assay.
  
  
  METHODS
  The D178N mutant prion PRNP was generated by the method of single site mutation. The mutant PRNP gene was inserted into plasmids of pET24. The full and N-truncated recombinant human prion proteins were expressed and purified. The fibril formations of these proteins were real-time monitored by the method of RT-QuIC. The ability to resist proteinase K (PK) of these fibrils was analyzed.
  
  
  RESULTS
  We succeed to construct human PrP-D178N plamids. The N-truncated human prion protein with D178N (PrP90-231-D178N) can convert spontaneously in RT-QuIC, while full length of human prion D178N protein (PrP23-231-D178N) fails to convert spontaneously. The spontaneously generated fibril has been domenstrated it is partily PK-resistant.
  
  
  CONCLUSIONS
  The N-terminal of prion protein (23-90) plays an important role for the D178N mutant protein spontaneously conversion, which provide the clues for study the pathogenesis of genetic CJD.
authors.affiliation: State Key Laboratory for Infectious Disease Prevention and Control, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing 100052, China. chenchengao@hotmail.com
authors.forename: Chen
authors.initials: C
authors.lastname: Gao
authors.forename: Ke
authors.initials: K
authors.lastname: Ren
authors.forename: Long-Zhu
authors.initials: LZ
authors.lastname: Li
authors.forename: Hui-Ying
authors.initials: HY
authors.lastname: Jiang
authors.forename: Cao
authors.initials: C
authors.lastname: Chen
authors.forename: Jin
authors.initials: J
authors.lastname: Zhang
authors.forename: Jun
authors.initials: J
authors.lastname: Han
authors.forename: Xiao-Ping
authors.initials: XP
authors.lastname: Dong
mesh_terms: D007562:Creutzfeldt-Jakob Syndrome / Q000209:etiology; D006801:Humans; D050505:Mutant Proteins / Q000235:genetics*; D021141:Nucleic Acid Amplification Techniques / Q000379:methods*; D011328:Prions / Q000235:genetics*
chemical_list: D050505:Mutant Proteins; D011328:Prions