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Related Experiment Videos

Lysosomal Quality Control in Prion Diseases.

Priyanka Majumder1, Oishee Chakrabarti2

  • 1Biophysics and Structural Genomics Division, Saha Institute of Nuclear Physics, Sector-1, Block-AF, Bidhannagar, Kolkata, West Bengal, 700064, India.

Molecular Neurobiology
|April 20, 2017
PubMed
Summary

Prion diseases involve misfolded prion proteins (PrP) damaging neurons. This review highlights how the lysosomal quality control pathway is crucial in these neurodegenerative disorders.

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Pathology

Background:

  • Prion diseases are neurodegenerative disorders caused by misfolded prion proteins (PrP).
  • These diseases, including Creutzfeldt-Jakob disease and bovine spongiform encephalopathy, are characterized by brain vacuolation.
  • Aberrant PrP forms, such as PrPSc and CtmPrP, are implicated in neuronal dysfunction and death.

Purpose of the Study:

  • To review the role of the lysosomal quality control pathway in prion diseases.
  • To explore how aberrant PrP isoforms interact with intracellular quality control mechanisms.
  • To emphasize the significance of the autophagosomal-lysosomal pathway in prion pathogenesis.

Main Methods:

  • Literature review focusing on prion diseases and cellular quality control.
Keywords:
AutophagosomeESCRT pathwayLysosomePrP

Related Experiment Videos

  • Analysis of the interplay between misfolded PrP and the lysosomal degradation system.
  • Examination of the involvement of ESCRT pathway proteins in prion disease phenotypes.

    • Main Results:

    • Lysosomal quality control machinery is a primary target for disease-causing PrP isoforms.
    • The autophagosomal-lysosomal pathway is impaired in prion diseases, modulating neurodegeneration.
    • ESCRT pathway proteins are involved in the manifestation of prion disease phenotypes.

    Conclusions:

    • The lysosomal quality control pathway plays a significant role in the pathogenesis of transmissible and familial prion diseases.
    • Understanding these pathways offers potential therapeutic targets for neurodegenerative conditions.
    • Aberrant PrP metabolism and its interaction with cellular quality control are key to disease progression.