Encephalopathy spongiform

Chemistry and molecular biology of transmissible spongiform encephalopathies 97AG(E)1674. 

Appendices This section is most likely to contain additional data associated with biological-based products. It should contain information as regards the facilities and equipment used for the manufacture of biotech products. Assessment of the risk of contamination from adventitious agents such as transmissible spongiform encephalopathy agents (TSEs), bacteria, mycoplasma, fungi or viruses should also be provided. Additional information on novel excipients that have not been used before should also be included in this section. 

D. Specific measures concerning prevention of transmission of animal Spongiform Encephalopathies (TSEs) (if applicable)... 

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. 

If the source of the infechon persists, after onset, then the incidence of new cases is maintained at a level which is commensurate with the infechvity of the pathogen and the ffequeney of exposure of individuals. In this manner, if cases of the variant Creutzfeldt-Jacob disease (vCJD), first recognized in the mid-1990s, relates to human exposure to bovine spongiform encephalopathy-infected beef in the early 1980s, then... 

Brown, P., Goldfarb, L.G. and Gajdusek, D.C. (1991b). The new biology of spongiform encephalopathy infectious amyloidosis with a genetic twist. Lancet 337, 1019-1022. 

Western blot A method to detect protein in a given sample of tissue homogenate or extract. It uses gel electrophoresis to separate denatured proteins by mass. Some diagnostic applications for the Western blot include Lyme disease, bovine spongiform encephalopathy, and human immunodeficiency virus (HIV) (it is considered the gold standard for HIV diagnostic testing). 

Where affected excipients are used—particularly when of bovine origin—including lactose, gelatin, stearic acid/stearates, etc., a statement of compliance with the current requirements relating to transmissible spongiform encephalopathies is included in the EPARs. 

Plasma cell disorders Plasma cell disorders Inflammation-associated, familial Mediterranean fever Familial amyloidotic neuropathy, systemic senile amyloidosis Dialysis-associated amyloidosis Familial amyloidotic neuropathy, aortic amyloidosis Familial systemic amyloidosis Familial systemic amyloidosis Familial systemic amyloidosis Familial cerebral hemorrhage with amyloidosis Sporadic and familial Alzheimer s disease, familial cerebral hemorrhage with amyloidosis Spongiform encephalopathies C-cell thyroid tumors Insulinoma, type II diabetes Atrial amyloidosis Prolactinomas pituitary amyloidosis Iatrogenic amyloidosis Corneal amyloidosis ... 

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

Amouyel P, Vidal O, Launay JM, La-pianche JL. The apolipoprotein E alleles as major susceptibility factors for Creutz-feldt-Jakob disease. The French Research Group on Epidemiology of Human Spongiform Encephalopathies. Lancet 1994 344[8933] 1315—1318. 

In addition, the regulatory authorities are concerned about contamination with viruses and prions, such as the causative agent of bovine spongiform encephalopathy (BSE), which could be present in mammalian cell cultures. It is necessary to... 

Suggested Alternatives for Differential Diagnosis Sheep Scrapie, pregnancy toxemia, hypocalcemia, tetanus, listeriosis, tick pyemia, hypocuprosis, rabies, hydatid disease, and various plant poisons. Cattle Malignant catarrhal fever, listeriosis, pseudorabies, bovine spongiform encephalopathy, rabies, hypomagnesemia, hypocalcemia, acute lead poisoning, and certain plant poisons. 

Minghetti,L., Greco,A., Cardone, F., Puopolo,M.,Ladogana, A. and Almonti, S. Increased brain synthesis of prostaglandin E2 and F2-isoprostane in human and experimental transmissible spongiform encephalopathies.. Neuropathol. Exp. Neurol. 59 866-871, 2000. 

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. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

Bruce, M. etal. Transmission of bovine spongiform encephalopathy and scrapie to mice Strain variation and the species barrier. Philos. Trans. R. Soc. Bond. [Biol] 343 405-411, 1994. 

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