The cellular and pathologic prion protein

Gill, Andrew and Castle, Andrew R. (2018) The cellular and pathologic prion protein. In: Human prion dIseases. Handbook of Clinical Neurology . Elsevier. ISBN 9780444639455

Full text not available from this repository.

Item Type:Book Section
Item Status:Live Archive


The cellular prion protein, PrPC, is a small, cell-surface glycoprotein with a function that is currently somewhat ill-defined. It is also the key molecule involved in the family of neurodegenerative disorders called transmissible spongiform encephalopathies, which are also known as prion diseases. The misfolding of PrPC to a conformationally-altered isoform, designated PrPSc, is the main molecular process involved in pathogenesis and appears to precede many other pathological and clinical manifestations of disease, including neuronal loss, astrogliosis and cognitive loss. PrPSc is also believed to be the major component of the infectious “prion”, the agent responsible for disease transmission, and preparations of this protein can cause prion disease when inoculated into a naïve host. Thus, understanding the biochemical and biophysical properties of both PrPC and PrPSc, and ultimately the mechanisms of their interconversion, are critical if we are to understand prion disease biology. Although entire books could be devoted to research pertaining to the protein, herein we briefly review the state of knowledge of prion biochemistry, including consideration of prion protein structure, function, misfolding and dysfunction.

Keywords:prion protein, misfolding, conformational conversion, toxicity, glycosylation, membrane anchor, PRNP, amyloid, prion function, cofactors
Subjects:A Medicine and Dentistry > A300 Clinical Medicine
Divisions:College of Science
ID Code:31412
Deposited On:18 Apr 2018 13:51

Repository Staff Only: item control page