Transmissible 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. 

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. 

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. 

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

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. 

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. 

Crevenakova, L. et al. (2002). Factor VIII and transmissible spongiform encephalopathy the case for safety. Haemophilia 8(2), 63—75. 

WHO (1997), Medicinal and other products and human and animal transmissible spongiform encephalopathies Memorandum from a WHO meeting, Bull. World Health Org., 75(6), 505-513. 

Rowlands SS. Transmissible spongiform encephalopathy and medicinal products in the United States and Europe. Drug Info J 2001 35 993-1001. 

Scallet A. C., Carp R. I., and Ye X. (2003). Pathophysiology of transmissible spongiform encephalopathies. Curr. Med. Chem. - Immunol. Endo. Metab. Agents 3 171-184. 

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. 

WHO (2003), Working Group on International Reference Materials for Diagnosis and Study of TSEs. Guidelines on Transmissible Spongiform Encephalopathies in Relation to Biological and Pharmaceutical Products, Geneva. Available at http // www.who.int/bloodproducts/publications/en/WHO TSE 2003.pdf (accessed October 2006). 

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