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Isolation of Soluble and Insoluble PrP Oligomers in the Normal Human Brain
11:29

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Published on: October 3, 2012

Loss of functional prion protein: a role in prion disorders?

D R Borchelt1, S S Sisodia

  • 1Department of Pathology, The Johns Hopkins University School of Medicine, 720 Rutland Avenue, 558 Ross Building, Baltimore, MD 21205, USA. drbor@welchlink.welch.jhu.edu

Chemistry & Biology
|August 1, 1996
PubMed

Insights

Mice lacking the prion protein (PrP) sometimes show prion disease symptoms. However, evidence suggests that losing functional PrP may not be the primary cause of these neurodegenerative disorders.

Area of Science:

  • Neuroscience
  • Molecular Biology
  • Protein Chemistry

Background:

  • The prion protein (PrP) is crucial for understanding prion disorders.
  • Research into PrP's normal function and disease mechanisms is ongoing.
  • Mice models are essential for studying PrP's role in health and disease.

Purpose of the Study:

  • To investigate the normal function of the prion protein (PrP).
  • To determine PrP's role in the pathogenesis of prion diseases.
  • To evaluate whether the absence of functional PrP causes prion-related symptoms.

Main Methods:

  • Generation of genetically modified mice lacking the PrP gene.
  • Phenotypic analysis of PrP-null mice to observe neurological and behavioral changes.
  • Experimental evaluation of alternative factors in prion disease development.

Main Results:

  • Some PrP-null mice exhibited symptoms resembling prion diseases.
  • Contradictory findings suggest PrP loss alone might not initiate these symptoms.
  • Further research is needed to identify the instigating factors in prion disorders.

Conclusions:

  • The precise function of PrP in normal physiology requires further elucidation.
  • The direct causal link between PrP absence and prion disease initiation is questionable.
  • Alternative molecular mechanisms may underlie the development of prion diseases.

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