전체메뉴
검색
Article Search

JMB Journal of Microbiolog and Biotechnology

QR Code QR Code

Related articles in JMB

More Related Articles

Article

J. Microbiol. Biotechnol. 2007; 17(7): 1059-1070

Published online July 28, 2007

Copyright © The Korean Society for Microbiology and Biotechnology.

Prions and Prion Diseases: Fundamentals and Mechanistic Details

Ryou, Chongsuk *

Sanders Brown Center on Aging, Department of Microbiology, Immunology and Molecular Genetics, College of Medicine,University of Kentucky, 800 Rose St. HSRB-326, Lexington, KY 40536, U.S.A.

Abstract

Prion diseases, often called transmissible spongiform encephalopathies (TSEs), are infectious diseases that accompany neurological dysfunctions in many mammalian hosts. Prion diseases include Creutzfeldt-Jakob disease (CJD) in humans, bovine spongiform encephalopathy (BSE, "mad cow disease") in cattle, scrapie in sheep, and chronic wasting disease (CWD) in deer and elks. The cause of these fatal diseases is a proteinaceous pathogen termed prion that lacks functional nucleic acids. As demonstrated in the BSE outbreak and its transmission to humans, the onset of disease is not limited to a certain species but can be transmissible from one host species to another. Such a striking nature of prions has generated huge concerns in public health and attracted serious attention in the scientific communities. To date, the potential transmission of prions to humans via foodborne infection and iatrogenic routes has not been alleviated. Rather, the possible transmission of human to human or cervids to human aggravates the terrifying situation across the globe. In this review, basic features about prion diseases including clinical and pathological characteristics, etiology, and transmission of diseases are described. Based on recently accumulated evidences, the molecular and biochemical aspects of prions, with an emphasis on the molecular interactions involved in prion conversion that is critical during prion replication and pathogenesis, are also addressed.

Keywords: Prion disease, prion, PrPC, PrPSc, species barrier, conformational conversion, prion replication, prion pathogenesis