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Inherited Forms of Creutzfeldt-Jakob Disease

Inherited Forms of Creutzfeldt-Jakob Disease CJD Creutzfeldt-Jakob Disease PRNP prion protein gene Introduction Protein infectious agent or “prion” is a concept developed by Stanley Prusiner in the 1980s of a form of infectious disease involving proteins rather than traditional pathogens. 1 More accurately, prion disease is a heterogeneous collection of neurodegenerative diseases characterized by alteration of a naturally occurring prion protein to a malformed protein with varying forms of presentation and transmission other than infection. Within the largest group of prion diseases—Creutzfeldt-Jakob disease—there are sporadic, familial, iatrogenic, and variant forms. CJD is an endemic disease with worldwide distribution; estimates of the incidence of sporadic or idiopathic CJD vary from 1 to 2 cases per million population annually. What is the Normal Function of the Prion Protein? The etiology of CJD entails a misfolding of the prion protein, but there is presently no consensus on a specific function for this protein. In light of its central role in a neurodegenerative disease, it is expected that the prion protein performs important functions in the brain, which, when disrupted, may lead to impairment of neurologic function. Prions are thought to serve in neuronal signaling as a membrane protein with cell signal-transduction properties. 2 Many authors argue http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Neuroradiology American Journal of Neuroradiology

Inherited Forms of Creutzfeldt-Jakob Disease

American Journal of Neuroradiology , Volume 34 (9): 1690 – Sep 1, 2013

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Publisher
American Journal of Neuroradiology
Copyright
Copyright © 2013 by the American Society of Neuroradiology.
ISSN
0195-6108
eISSN
1936-959X
DOI
10.3174/ajnr.A3580
pmid
23557959
Publisher site
See Article on Publisher Site

Abstract

CJD Creutzfeldt-Jakob Disease PRNP prion protein gene Introduction Protein infectious agent or “prion” is a concept developed by Stanley Prusiner in the 1980s of a form of infectious disease involving proteins rather than traditional pathogens. 1 More accurately, prion disease is a heterogeneous collection of neurodegenerative diseases characterized by alteration of a naturally occurring prion protein to a malformed protein with varying forms of presentation and transmission other than infection. Within the largest group of prion diseases—Creutzfeldt-Jakob disease—there are sporadic, familial, iatrogenic, and variant forms. CJD is an endemic disease with worldwide distribution; estimates of the incidence of sporadic or idiopathic CJD vary from 1 to 2 cases per million population annually. What is the Normal Function of the Prion Protein? The etiology of CJD entails a misfolding of the prion protein, but there is presently no consensus on a specific function for this protein. In light of its central role in a neurodegenerative disease, it is expected that the prion protein performs important functions in the brain, which, when disrupted, may lead to impairment of neurologic function. Prions are thought to serve in neuronal signaling as a membrane protein with cell signal-transduction properties. 2 Many authors argue

Journal

American Journal of NeuroradiologyAmerican Journal of Neuroradiology

Published: Sep 1, 2013

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