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Role of lipid in forming an infectious prion?

Fei Wang1, Jiyan Ma

  • 1Department of Molecular and Cellular Biochemistry, Ohio State University, 1645 Neil Ave., Columbus, OH 43210, USA. wang.797@osu.edu

Acta Biochimica Et Biophysica Sinica
|April 16, 2013
PubMed

Insights

Prion diseases are caused by infectious proteins. Recent studies confirm that lipids are crucial cofactors in forming these infectious prion agents, advancing the prion hypothesis.

Area of Science:

  • Neuroscience
  • Biochemistry
  • Infectious Diseases

Background:

  • Transmissible spongiform encephalopathies (TSEs), or prion diseases, are fatal neurodegenerative disorders.
  • The prion hypothesis proposes that an infectious protein conformer causes disease transmission.
  • Decades of research have centered on the nature of the infectious agent.

Purpose of the Study:

  • To review the chemical nature of the prion infectious agent.
  • To explore the role of lipid cofactors in prion infectivity.
  • To discuss outstanding questions in prion disease research.

Main Methods:

  • Review of existing scientific literature on prions and TSEs.
  • Analysis of studies generating prion infectivity from recombinant prion proteins.
  • Examination of evidence for cofactor involvement, particularly lipids.

Main Results:

  • Overwhelming evidence supports the prion hypothesis: a misfolded prion protein can induce normal prion proteins to misfold.
  • Recent studies demonstrate prion infectivity using purified, bacterially expressed recombinant prion protein.
  • Lipid or lipid-like molecules are indicated as essential cofactors in forming infectious prion agents.

Conclusions:

  • The prion hypothesis is strongly supported by recent experimental findings.
  • Lipids play a critical role in the formation and infectivity of prion agents.
  • Further research is needed to fully elucidate the chemical nature and mechanisms of prion infectivity.
Keywords:
lipidprionprion infectivityprion protein conversionrecombinant prion

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