TY  - JOUR
AU  - Kretzschmar, Hans
AU  - Tatzelt, Jörg
TI  - Prion disease: a tale of folds and strains.
JO  - Brain pathology
VL  - 23
IS  - 3
SN  - 1015-6305
CY  - Oxford
PB  - Wiley-Blackwell
M1  - DZNE-2020-03201
SP  - 321-332
PY  - 2013
AB  - Research on prions, the infectious agents of devastating neurological diseases in humans and animals, has been in the forefront of developing the concept of protein aggregation diseases. Prion diseases are distinguished from other neurodegenerative diseases by three peculiarities. First, prion diseases, in addition to being sporadic or genetic like all other neurodegenerative diseases, are infectious diseases. Animal models were developed early on (a long time before the advent of transgenic technology), and this has made possible the discovery of the prion protein as the infectious agent. Second, human prion diseases have true equivalents in animals, such as scrapie, which has been the subject of experimental research for many years. Variant Creutzfeldt-Jakob disease (vCJD) is a zoonosis caused by bovine spongiform encephalopathy (BSE) prions. Third, they show a wide variety of phenotypes in humans and animals, much wider than the variants of any other sporadic or genetic neurodegenerative disease. It has now become firmly established that particular PrP(Sc) isoforms are closely related to specific human prion strains. The variety of human prion diseases, still an enigma in its own right, is a focus of this article. Recently, a series of experiments has shown that the concept of aberrant protein folding and templating, first developed for prions, may apply to a variety of neurodegenerative diseases. In the wake of these discoveries, the term prion has come to be used for Aβ, α-synuclein, tau and possibly others. The self-propagation of alternative conformations seems to be the common denominator of these 'prions,' which in future, in order to avoid confusion, may have to be specified either as 'neurodegenerative prions' or 'infectious prions.'
KW  - Animals
KW  - Blood-Brain Barrier
KW  - Creutzfeldt-Jakob Syndrome: genetics
KW  - Creutzfeldt-Jakob Syndrome: pathology
KW  - Genome-Wide Association Study
KW  - Humans
KW  - Kuru: genetics
KW  - Kuru: pathology
KW  - PrPC Proteins: chemistry
KW  - PrPC Proteins: genetics
KW  - PrPC Proteins: toxicity
KW  - Prion Diseases: genetics
KW  - Prion Diseases: pathology
KW  - Prion Diseases: transmission
KW  - Protein Folding
KW  - Proteostasis Deficiencies: genetics
KW  - Proteostasis Deficiencies: pathology
KW  - PrPC Proteins (NLM Chemicals)
LB  - PUB:(DE-HGF)16
C6  - pmid:23587138
DO  - DOI:10.1111/bpa.12045
UR  - https://pub.dzne.de/record/136879
ER  -