Transmissible spongiform diseases

The transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative diseases characterized by spongiform changes, astrocytic gliomas, and neuronal loss resulting from the deposition of insoluble protein aggregates in neural cells. They include Creutzfeldt-Jakob disease in humans, scrapie in... 

Prions—protein particles that lack nucleic acid— cause fatal transmissible spongiform encephalopathies such as Creutzfeldt-Jakob disease, scrapie, and bovine spongiform encephalopathy. Prion diseases involve an altered secondary-tertiary strucmre of a namrally occurring protein, PrPc. When PrPc interacts with its pathologic isoform PrPSc, its conformation is transformed from a predominantly a-helical strucmre to the P-sheet strucmre characteristic of PrPSc. 

Foster J, Hunter N. Transmissible spongiform encephalopathies transmission, mechanism of disease, and persistence. Curr Opin Microbiol 1998 1 442-447. 

Pocchiari, M., Puopolo, M., Croes, E. A. et al. Predictors of survival in sporadic Creutzfeldt-Jakob disease and other human transmissible spongiform encephalopathies. Brain 127 2348-2359,2004. 

This working party considers the aspects of the manufacture and control of biotechnological and biological medicinal products and is also involved in the provision of scientific advice. Workshops on the applieation of assays for markers of transmissible spongiform encephalopathies (TSE) and on the potential risk of transmitting new variant Creutzfeld-Jakob disease (nv-CJD) through plasma-derived medicinal products have recently been held. 

Phenotypically, different neurodegenerative disorders, including AD, Parkinson disease, and prion diseases (transmissible spongiform encephalopathies), are... 

Creutzfeldt-Jacob disease—Human form of transmissible spongiform encephalopathy (TSE), a brain disease in which nerve cells of the brain are destroyed. Seen under a microscope, the brain tissue of people with TSE resembles a sponge. 

Mad cow disease—Bovine spongiform encephalopathy, or BSE, the form of transmissible spongiform encephalopathy (TSE) found in cattle. It is thought to be spread by consumption of brain tissue and caused by proteins called prions. 

Transmissible spongiform encephalopathies (TSEs)—Brain diseases transmitted from one animal to another. Under a microscope, the brain tissue of animals and people with TSEs resembles a sponge. TSEs include variant Creutzfeldt-Jacob disease (vCJD) in humans, scrapie in sheep and goats, and bovine spongiform encephalopathy (BSE) in cows (mad cow disease). These diseases are spread by consumption of brain tissue and are thought to be caused by prions, a kind of protein. 

Events or structures occurring proximal to the synapse. Transmissible spongiform encephalopathy is an example, together with Creutzfeldt-Jakob disease and bovine spongiform encephalopathy. Caused by abnormal proteins. 

In human populations, exposure to the BSE agent (probably in contaminated bovine-based food products) has been strongly linked to the 1996 appearance of a new transmissible spongiform encephalopathy of humans called variant Creutzfeldt-Jakob Disease (vCJD). 

Currently, there are some concerns regarding Transmissible Spongiform Encephalopathies (TSE) via animal-derived excipients such as gelatin. TSEs are caused by prions that are extremely resistant to heat and normal sterilization processes. TSEs have a very long incubation time with no cure and include diseases such as the following ... 

Two advisory committees of the US FDA, the Transmissible Spongiform Encephalopathies Advisory Committee and the Vaccines and Related Biologicals Product Advisory Committee, said at a joint meeting on 3 August 2000 that vaccines made from bovine-derived materials from countries with a known or uncertain risk of BSE carry only an infinitesimal risk of new variant Creutzfeldt-Jakob disease, and that no change in US immunization practice is indicated (Evans G, personal communication, 3 August 2000). 

Certain brain diseases of humans and animals known as proteinopathies are associated with the accumulation of misfolded proteins both inside and around neurons [1], Alzheimer s disease in humans is by far the most common member of this group. The prion diseases, exemplified by Creutzfeldt-Jakob disease (CJD) in man, scrapie in small ruminants, and bovine spongiform encephalopathy (BSE) in cattle, constitute a peculiar sub-group within proteinopathies by being experimentally transmissible [2], Because of this feature, together with the sponge-like appearance of vacuoles in affected brain areas, prion diseases are also known as Transmissible Spongiform Encephalopathies (TSEs). 

Jennelle CS, Samuel MD, Nolden CA et al (2009) Surveillance for transmissible spongiform encephalopathy in scavengers of white-tailed deer carcasses in the chronic wasting disease area of Wisconsin. J Toxicol Environ Health A 72 1018-1024... 

Der Trab ist auch eine Krankheit der Schaafe, und ist ansteckend. Sie schleppen sich lange, verzehren sich nach und nach, und zuletzt miissen sie sterben. These sentences are taken from an article published in 1759 [1] and describe two hallmarks of prion diseases or transmissible spongiform encephalopathies (TSEs, summarized in Table 1) The formation and transmission of an infectious particle and the invariably fatal course of these diseases. More than 200 years later a landmark discovery paved the way to study the pathogenesis of prion diseases at a molecular level. Prusiner and colleagues reported the identification of a protease-resistant protein in brain extracts, which co-purified with the infectious scrapie agent [18]. After the N-terminal amino acid sequence of the proteinase K (PK)-resistant core of the prion protein (PrP 27-30) was published in 1984 [19], two... 

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