Spongiform encephalopathy

Common Name(s)

Spongiform encephalopathy, Gerstmann Straussler Scheinker syndrome, Gerstmann-Straussler-Scheinker disease, Amyloidosis cerebral with spongiform encephalopathy

Gerstmann-Straussler-Scheinker disease is a neurodegenerative brain disorder which causes ataxia and dementia. It is almost always inherited and is found in only a few families around the world. Onset of the disease usually occurs between the ages of 35 and 55. Gerstmann-Straussler-Scheinker disease belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs) or prion diseases. Other TSEs include Creutzfeldt-Jakob disease, kuru, and fatal familial insomnia.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Spongiform encephalopathy" for support, advocacy or research.

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Spongiform encephalopathy" returned 84 free, full-text research articles on human participants. First 3 results:

Removal of transmissible spongiform encephalopathy prion from large volumes of cell culture media supplemented with fetal bovine serum by using hollow fiber anion-exchange membrane chromatography.
 

Author(s): Ming Li Chou, Andy Bailey, Tiffany Avory, Junji Tanimoto, Thierry Burnouf

Journal:

 

Cases of variant Creutzfeldt-Jakob disease in people who had consumed contaminated meat products from cattle with bovine spongiform encephalopathy emphasize the need for measures aimed at preventing the transmission of the pathogenic prion protein (PrPSc) from materials derived from ...

Last Updated: 16 Apr 2015

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The glycosylation status of PrPC is a key factor in determining transmissible spongiform encephalopathy transmission between species.
 

Author(s): Frances K Wiseman, Enrico Cancellotti, Pedro Piccardo, Kayleigh Iremonger, Aileen Boyle, Deborah Brown, James W Ironside, Jean C Manson, Abigail B Diack

Journal: J. Virol.. 2015 May;89(9):4738-47.

 

The risk of transmission of transmissible spongiform encephalopathies (TSE) between different species has been notoriously unpredictable because the mechanisms of transmission are not fully understood. A transmission barrier between species often prevents infection of a new host with ...

Last Updated: 4 Apr 2015

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Genotype-dependent molecular evolution of sheep bovine spongiform encephalopathy (BSE) prions in vitro affects their zoonotic potential.
 

Author(s): Zuzana Krejciova, Marcelo A Barria, Michael Jones, James W Ironside, Martin Jeffrey, Lorenzo González, Mark W Head

Journal: J. Biol. Chem.. 2014 Sep;289(38):26075-88.

 

Prion diseases are rare fatal neurological conditions of humans and animals, one of which (variant Creutzfeldt-Jakob disease) is known to be a zoonotic form of the cattle disease bovine spongiform encephalopathy (BSE). What makes one animal prion disease zoonotic and others not is ...

Last Updated: 20 Sep 2014

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Spongiform encephalopathy" returned 23 free, full-text review articles on human participants. First 3 results:

Laboratory activities involving transmissible spongiform encephalopathy causing agents: risk assessment and biosafety recommendations in Belgium.
 

Author(s): Amaya Leunda, Bernadette Van Vaerenbergh, Aline Baldo, Stefan Roels, Philippe Herman

Journal: Prion. ;7(5):420-33.

 

Since the appearance in 1986 of epidemic of bovine spongiform encephalopathy (BSE), a new form of neurological disease in cattle which also affected human beings, many diagnostic and research activities have been performed to develop detection and therapeutic tools. A lot of progress ...

Last Updated: 13 Jan 2014

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Experimental interspecies transmission studies of the transmissible spongiform encephalopathies to cattle: comparison to bovine spongiform encephalopathy in cattle.
 

Author(s): Amir N Hamir, Marcus E Kehrli, Robert A Kunkle, Justin J Greenlee, Eric M Nicholson, Jürgen A Richt, Janice M Miller, Randall C Cutlip

Journal: J. Vet. Diagn. Invest.. 2011 May;23(3):407-20.

 

Prion diseases or transmissible spongiform encephalopathies (TSEs) of animals include scrapie of sheep and goats; transmissible mink encephalopathy (TME); chronic wasting disease (CWD) of deer, elk and moose; and bovine spongiform encephalopathy (BSE) of cattle. The emergence of BSE ...

Last Updated: 12 Sep 2011

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[Prions: where do we stand 20 years after the appearance of bovine spongiform encephalopathy?].
 

Author(s): Carole Crozet, Sylvain Lehmann

Journal: Med Sci (Paris). 2007 Dec;23(12):1148-57.

 

Bovine spongiform encephalopathy (BSE) is a transmissible spongiform encephalopathy (TSE) identified twenty years ago in the British cattle herds. Creutzfeldt-Jakob disease (CJD) is a TSE that occurs in humans. In 1996, scientists found a possible link between BSE and a new variant ...

Last Updated: 24 Dec 2007

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Enhanced CJD Surveillance in the Older Population
 

Status: Recruiting

Condition Summary: Prion Disease; Dementia

 

Last Updated: 26 Jan 2016

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The Role of the Coagulation Pathway at the Synapse in Prion Diseases
 

Status: Not yet recruiting

Condition Summary: Creutzfeldt-Jakob Syndrome

 

Last Updated: 23 Jun 2015

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Last Updated: 30 Mar 2015

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