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López-Pérez Ó, Badiola JJ, Bolea R, et al. An Update on Autophagy in Prion Diseases. Front Bioeng Biotechnol. 2020;8:975doi: 10.3389/fbioe.2020.00975.
López-Pérez, �. �., Badiola, J. J., Bolea, R., Ferrer, I., Llorens, F., & Martín-Burriel, I. (2020). An Update on Autophagy in Prion Diseases. Frontiers in bioengineering and biotechnology, 8975. https://doi.org/10.3389/fbioe.2020.00975
López-Pérez, Óscar, et al. "An Update on Autophagy in Prion Diseases." Frontiers in bioengineering and biotechnology vol. 8 (2020): 975. doi: https://doi.org/10.3389/fbioe.2020.00975
López-Pérez Ó, Badiola JJ, Bolea R, Ferrer I, Llorens F, Martín-Burriel I. An Update on Autophagy in Prion Diseases. Front Bioeng Biotechnol. 2020 Aug 27;8:975. doi: 10.3389/fbioe.2020.00975. eCollection 2020. PMID: 32984276; PMCID: PMC7481332.
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Front Bioeng Biotechnol. 2020 Aug 27;8:975. doi: 10.3389/fbioe.2020.00975. eCollection 2020.

An Update on Autophagy in Prion Diseases.

Frontiers in bioengineering and biotechnology

Óscar López-Pérez, Juan José Badiola, Rosa Bolea, Isidro Ferrer, Franc Llorens, Inmaculada Martín-Burriel

Affiliations

  1. Laboratorio de Genética Bioquímica (LAGENBIO), Instituto Agroalimentario de Aragón-IA2, Instituto de Investigación Sanitaria Aragón-IISA, Universidad de Zaragoza, Zaragoza, Spain.
  2. Centro de Encefalopatías y Enfermedades Transmisibles Emergentes (CEETE), Instituto Agroalimentario de Aragón-IA2, Instituto de Investigación Sanitaria Aragón-IISA, Universidad de Zaragoza, Zaragoza, Spain.
  3. Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Instituto Carlos III, L'Hospitalet de Llobregat, Barcelona, Spain.
  4. Instituto de Investigación Biomédica de Bellvitge (IDIBELL), L'Hospitalet de Llobregat, Barcelona, Spain.
  5. Departamento de Patología y Terapéutica Experimental, Universidad de Barcelona, L'Hospitalet de Llobregat, Barcelona, Spain.
  6. Department of Neurology, Clinical Dementia Center and National Reference Center for CJD Surveillance, University Medical School, Göttingen, Germany.
  7. Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED), Instituto Carlos III, Zaragoza, Spain.

PMID: 32984276 PMCID: PMC7481332 DOI: 10.3389/fbioe.2020.00975

Abstract

Autophagy is a dynamic intracellular mechanism involved in protein and organelle turnover through lysosomal degradation. When properly regulated, autophagy supports normal cellular and developmental processes, whereas defects in autophagic degradation have been associated with several pathologies, including prion diseases. Prion diseases, or transmissible spongiform encephalopathies (TSE), are a group of fatal neurodegenerative disorders characterized by the accumulation of the pathological misfolded isoform (PrP

Copyright © 2020 López-Pérez, Badiola, Bolea, Ferrer, Llorens and Martín-Burriel.

Keywords: Creutzfeldt-Jakob disease; LC3; autophagy; neurodegenerative diseases; p62; prion diseases; scrapie; therapies

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