Header

UZH-Logo

Maintenance Infos

Gerstmann–Sträussler–Scheinker Disease


Budka, H; Kovacs, G G (2013). Gerstmann–Sträussler–Scheinker Disease. In: Maloy, Stanley; Hughes, Kelly. Brenner's Encyclopedia of Genetics, 2nd Edition. New York: Elsevier B.V., 327-329.

Abstract

Gerstmann–Sträussler–Scheinker (GSS) disease is a rare human neurodegenerative disease characterized clinically by early cerebellar ataxia and later progressive dementia, and neuropathologically by abundant multicentric amyloid plaques composed of the prion protein (PrP). It belongs to the genetic prion diseases such as genetic or familial Creutzfeldt–Jakob disease (gCJD/fCJD) and fatal familial insomnia (FFI), and is associated with specific missense mutations in the PrP gene (PRNP).

Abstract

Gerstmann–Sträussler–Scheinker (GSS) disease is a rare human neurodegenerative disease characterized clinically by early cerebellar ataxia and later progressive dementia, and neuropathologically by abundant multicentric amyloid plaques composed of the prion protein (PrP). It belongs to the genetic prion diseases such as genetic or familial Creutzfeldt–Jakob disease (gCJD/fCJD) and fatal familial insomnia (FFI), and is associated with specific missense mutations in the PrP gene (PRNP).

Statistics

Altmetrics

Additional indexing

Item Type:Book Section, not refereed, further contribution
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Institute of Neuropathology
Dewey Decimal Classification:570 Life sciences; biology
610 Medicine & health
Language:English
Date:February 2013
Deposited On:18 Dec 2013 16:21
Last Modified:05 Apr 2016 17:17
Publisher:Elsevier B.V.
ISBN:9780123749840

Download

Full text not available from this repository.