Transmissible spongiform encephalopathies (TSEs, also known as prion diseases) are a group of progressive conditions that affect the brain and nervous system of humans and animals and are transmitted by prions. Mental and physical abilities deteriorate and myriad tiny holes appear in the cortex causing it to appear like a sponge (hence 'spongiform') when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen over time. Prion diseases of humans include classic Creutzfeldt-Jakob disease, new variant Creutzfeldt-Jakob disease (a human disorder related to mad cow disease), Gerstmann-Sträussler-Scheinker syndrome, fatal insomnia and kuru. These conditions form a spectrum of diseases with overlapping signs and symptoms.

Unlike other kinds of infectious disease which are spread by microbes, the infectious agent in TSEs is a specific protein called prion protein. Misshaped prion proteins carry the disease between individuals and cause deterioration of the brain. TSEs are unique diseases in that they can be inherited, occur spontaneously ("sporadic" TSE) or can be spread through infection (Collinge, 2001). Most TSEs are sporadic and occur in an animal with no prion protein mutation. Inherited TSE occurs in animals carrying a rare mutant prion allele, which expresses prion proteins that contort by themselves into the disease-causing conformation. Transmission occurs when healthy animals consume tainted tissues from others with the disease. In recent times a type of TSE called bovine spongiform encephalopathy (BSE) spread in cattle in an epidemic fashion. This occurred because cattle were fed the processed remains of other cattle, a practice now banned in many countries. The epidemic could have begun with just one cow with sporadic disease.

Prions cannot be transmitted through the air or through touching or most other forms of casual contact. However, they may be transmitted through contact with infected tissue, body fluids, or contaminated medical instruments. Normal sterilization procedures such as boiling or irradiating materials fail to render the pathogens non-infective.

More on [ Transmissible spongiform encephalopathy ]

Prion Diseases - Twitter Search

Family's inherited condition links prion diseases, Alzheimer's. http://mednews.wustl.edu/news/page/normal/15213.html
UCSFMAC (UCSF MAC) Fri, 18 Dec 2009 18:37:57 -0000
Family's inherited condition links prion diseases, Alzheimer's. http://mednews.wustl.edu/news/page/normal/15213.html
RT @DDOpinion #Glimepiride Potential for Treatment of #Prion Diseases http://bit.ly/8eHxUB
Chemical_Alley (Jay) Wed, 16 Dec 2009 09:28:36 -0000
RT @DDOpinion #Glimepiride Potential for Treatment of #Prion Diseases http://bit.ly/8eHxUB
Glimepiride Potential for Treatment of Prion Diseases http://bit.ly/8eHxUB
DDOpinion (Bill Harris) Wed, 16 Dec 2009 08:48:36 -0000
Glimepiride Potential for Treatment of Prion Diseases http://bit.ly/8eHxUB
R1 Family's inherited condition links prion diseases, Alzheimer's http://nxy.in/d8vmz
anoushka89 (anoushka) Tue, 15 Dec 2009 20:44:04 -0000
R1 Family's inherited condition links prion diseases, Alzheimer's http://nxy.in/d8vmz
Family's inherited condition links prion diseases, Alzheimer's http://nxy.in/d8vmz
anoushka89 (anoushka) Tue, 15 Dec 2009 06:39:01 -0000
Family's inherited condition links prion diseases, Alzheimer's http://nxy.in/d8vmz
Saw amazing lecture by nobel laureate S. Prusiner on why he believes Parkinson's and Alzheimer's are prion diseases.
pnschmidt (Peter Schmidt) Mon, 14 Dec 2009 00:34:45 -0000
Saw amazing lecture by nobel laureate S. Prusiner on why he believes Parkinson's and Alzheimer's are prion diseases.

Subscribe to Prion_Diseases RSS feed