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Article

Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix.

Nature medicine - 1 Jul 1997

Muramoto T, DeArmond S J, Scott M, Telling G C, Cohen F E, Prusiner S B

Abstract excerpt

Mice were constructed carrying prion protein (PrP) transgenes with individual regions of putative secondary structure deleted. Transgenic mice with amino-terminal regions deleted remained healthy at >400 days of age, whereas those with either of carboxy-terminal alpha-helices deleted spontaneously developed fatal CNS illnesses similar to neuronal storage diseases. Deletion of either C-terminal helix resulted in...

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Source

Identifiers

PMID: 9212101
DOI: 10.1038/nm0797-750

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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