In:
Science, American Association for the Advancement of Science (AAAS), Vol. 279, No. 5352 ( 1998-02-06), p. 827-834
Kurzfassung:
At the endoplasmic reticulum membrane, the prion protein (PrP) can be synthesized in several topological forms. The role of these different forms was explored with transgenic mice expressing PrP mutations that alter the relative ratios of the topological forms. Expression of a particular transmembrane form (termed Ctm PrP) produced neurodegenerative changes in mice similar to those of some genetic prion diseases. Brains from these mice contained Ctm PrP but not PrP Sc , the PrP isoform responsible for transmission of prion diseases. Furthermore, in one heritable prion disease of humans, brain tissue contained Ctm PrP but not PrP Sc . Thus, aberrant regulation of protein biogenesis and topology at the endoplasmic reticulum can result in neurodegeneration.
Materialart:
Online-Ressource
ISSN:
0036-8075
,
1095-9203
DOI:
10.1126/science.279.5352.827
Sprache:
Englisch
Verlag:
American Association for the Advancement of Science (AAAS)
Publikationsdatum:
1998
ZDB Id:
128410-1
ZDB Id:
2066996-3
ZDB Id:
2060783-0
SSG:
11
