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Human prion diseases.

M S Palmer1, J Collinge

  • 1Department of Biochemistry and Molecular Genetics, St Mary's Hospital Medical School, London, UK.

Current Opinion in Neurology and Neurosurgery
|December 1, 1992
PubMed
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Advances in prion disease research suggest an abnormal protein isoform is the transmissible agent. Direct gene testing now diagnoses inherited prion diseases, clarifying their dual nature.

Area of Science:

  • Neuroscience
  • Genetics
  • Infectious Diseases

Background:

  • Prion diseases are a group of fatal neurodegenerative disorders.
  • The transmissible agent is increasingly accepted to be an abnormal host protein isoform.
  • Understanding the link between inherited and transmissible forms remains a challenge.

Purpose of the Study:

  • To summarize recent advances in prion disease research.
  • To highlight the role of molecular genetics in understanding prion disease phenotypes.
  • To address the paradox of diseases being both inherited and transmissible.

Main Methods:

  • Review of recent scientific literature on prion diseases.
  • Analysis of molecular genetic studies.
  • Discussion of diagnostic advancements.

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Main Results:

  • Significant progress in understanding prion disease mechanisms.
  • Growing consensus on the prion protein hypothesis.
  • Identification of direct gene testing for inherited prion disease diagnosis.

Conclusions:

  • The abnormal prion protein isoform is likely the infectious agent.
  • Molecular genetics has expanded the known spectrum of prion diseases.
  • The inheritance and transmissibility of prion diseases are becoming better understood.