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Artificial strain of human prions created in vitro

Chae Kim1, Xiangzhu Xiao2, Shugui Chen2,3

  • 1Department of Pathology, Case Western Reserve University, 2085 Adelbert Rd, Cleveland, OH, 44106, USA.

Nature Communications
|June 6, 2018
PubMed

Insights

Researchers synthesized infectious human prions from recombinant prion protein. These prions caused neurological disease in mice, suggesting structural changes in prion protein influence infectivity and disease characteristics.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Infectious Diseases

Background:

  • Sporadic Creutzfeldt-Jakob disease (sCJD) is the most common human prion disease.
  • The molecular mechanisms governing sCJD transmissibility remain largely unknown.
  • Understanding prion infectivity is crucial for developing therapeutic strategies.

Purpose of the Study:

  • To synthesize infectious human prions from recombinant human prion protein.
  • To investigate the biological and structural characteristics of these synthetic prions.
  • To explore the role of structural modifications in prion infectivity and host targeting.

Main Methods:

  • Recombinant human prion protein expressed in bacteria was used.
  • Synthesis was seeded with sCJD MM1 prions and the cofactor ganglioside GM1.
  • Infectivity was tested in transgenic mice expressing non-glycosylated human prion protein.

Main Results:

  • Synthetic human prions were successfully created and were infectious to transgenic mice.
  • Mice exhibited neurological dysfunction after 459 days (first passage) and 224 days (second passage).
  • Neuropathology and biophysical profiling indicated a novel, neurotoxic human prion strain.

Conclusions:

  • Subtle structural changes in prion protein domains, potentially including posttranslational modifications, are critical determinants of prion infectivity.
  • These structural variations influence prion host range and specific brain structure targeting.
  • The study provides insights into the molecular mechanisms of human prion disease transmission and pathogenesis.

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