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Protein Misfolding Cyclic Amplification

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Prions and Diseases

Abstract

Prion diseases are caused by a conformational conversion of the cellular prion protein (PrPC) to a pathological conformer (PrPSc). The “prion-only” hypothesis suggests that PrPSc is the infectious agent that propagates the disease acting as a template for the conversion of PrPC. In 2001, we developed a novel in vitro technique, called Protein misfolding cyclic amplification (PMCA), which mimics this pathological process in an accelerated way. Thereby, minimal amount of PrPSc can be amplified to several millions fold, providing an important tool for diagnosis and investigation of prion biology, and the molecular mechanism of prion conversion. PMCA also offers a great platform for the study and amplification of the protein misfolding process associated with other neurodegenerative disorders, such as Alzheimer’s and Parkinson’s diseases.

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Author information

Authors and Affiliations

  1. Department of Neurology, Mitchell Center for Alzheimer’s disease and Related Brain Disorders, University of Texas Houston Medical School, Houston, TX, USA

    Fabio Moda Ph.D., Sandra Pritzkow Ph.D. & Claudio Soto Ph.D.

Authors
  1. Fabio Moda Ph.D.
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  2. Sandra Pritzkow Ph.D.
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  3. Claudio Soto Ph.D.
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Editors and Affiliations

  1. , Department of Pathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Wen-Quan Zou

  2. , Department of Neuropathology, Case Western Reserve University School o, 2085 Adelbert Road, Cleveland, 44106, Ohio, USA

    Pierluigi Gambetti

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Moda, F., Pritzkow, S., Soto, C. (2013). Protein Misfolding Cyclic Amplification. In: Zou, WQ., Gambetti, P. (eds) Prions and Diseases. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-5305-5_6

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