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Bovine Spongiform Encephalopathy (Mad Cow Disease)

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A transmissible spongiform encephalopathy of cattle associated with abnormal prion proteins in the brain. Affected animals develop excitability and salivation followed by ATAXIA. This disorder has been associated with consumption of SCRAPIE infected ruminant derived protein. This condition may be transmitted to humans, where it is referred to as variant or new variant CREUTZFELDT-JAKOB SYNDROME. (Vet Rec 1998 Jul 25;143(41):101-5)

Also Known As:

Mad Cow Disease; Encephalopathy, Bovine Spongiform; BSE (Bovine Spongiform Encephalopathy); Encephalitis, Bovine Spongiform; BSEs (Bovine Spongiform Encephalopathy); Bovine Spongiform Encephalitis; Mad Cow Diseases; Spongiform Encephalopathy, Bovine

Networked: 394 relevant articles (6 outcomes, 26 trials/studies) for this Disease, Comments

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Scrapie
2.	Infection
3.	Creutzfeldt-Jakob Syndrome (Creutzfeldt-Jakob Disease)
4.	Kuru
5.	Hemorrhage

Experts

1.	Collinge, John: 11 articles (03/2011 - 04/2003)
2.	Prusiner, Stanley B: 8 articles (09/2011 - 03/2002)
3.	Yokoyama, Takashi: 8 articles (04/2011 - 05/2002)
4.	Mohri, Shirou: 6 articles (04/2011 - 04/2007)
5.	Masujin, Kentaro: 5 articles (04/2011 - 06/2007)
6.	Imamura, Morikazu: 4 articles (04/2011 - 01/2009)
7.	Mead, Simon: 4 articles (02/2011 - 06/2006)
8.	DeArmond, Stephen J: 4 articles (04/2009 - 03/2002)
9.	Safar, Jiri G: 3 articles (09/2011 - 11/2002)
10.	Giles, Kurt: 3 articles (09/2011 - 11/2008)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Bovine Spongiform Encephalopathy:

1.	Amyloid (Amyloid Fibrils)IBA 12/15/2010 - "These results quantitatively inform reaction-based theories of amyloid fiber formation and have implications for neurodegenerative, protein conformation ailments including Alzheimer's disease and bovine spongiform encephalopathy." 08/01/1995 - "Regional amyloid protein pattern analysis of brain and spinal cord may help to distinguish sporadic from environmentally acquired infections, as for example, cases of human disease suspected to have arisen from exposure to sheep or cows infected with scrapie or bovine spongiform encephalopathy." 09/16/2002 - "Today several diseases are known, for example Alzheimer's disease, Creutzfeldt-Jakob disease, mad cow disease, in which amyloid formation is involved. " 10/01/1995 - "Using an improved extraction method and Western blot detection, the disease-specific amyloid was found in various parts of the central nervous system of hamsters orally infected with scrapie, of squirrel monkeys orally infected with kuru, sporadic Creutzfeldt-Jakob disease (CJD) and scrapie, of human patients with sporadic CJD, of a sheep with natural scrapie and of a cow with bovine spongiform encephalopathy (BSE). " 01/19/2007 - "The mammalian prion, PrP(Sc), responsible for neurodegenerative diseases like bovine spongiform encephalopathy (BSE; "mad cow" disease) and Creutzfeldt-Jakob's disease, appears to be a beta-sheet-rich amyloid conformation of PrP(c) that converts PrP(c) into PrP(Sc). " Order ALL the reference details at left...
2.	ParaffinIBA 05/01/2011 - "The paraffin-embedded tissue (PET) blot was modified for use as a tool to differentiate between classical scrapie and experimental bovine spongiform encephalopathy (BSE) in sheep. " 05/01/2011 - "Paraffin-embedded tissue blot as a sensitive method for discrimination between classical scrapie and experimental bovine spongiform encephalopathy in sheep." 01/01/1994 - "The efficacy of three pretreatment techniques for the detection of prion protein (PrP) in formalin-fixed, paraffin-embedded bovine spongiform encephalopathy (BSE)--affected brain tissue were compared using automated image analysis. " 05/01/2010 - "To initially evaluate the immunoAT method, the presence or absence of prion protein (PrP(sc)) was determined in formalin-fixed and paraffin-embedded sections of the medulla oblongata of cattle which had been under active surveillance for bovine spongiform encephalopathy. " 09/01/2010 - "Detection of PrP(Sc) in formalin-fixed, paraffin-embedded tissue by Western blot differentiates classical scrapie, Nor98 scrapie, and bovine spongiform encephalopathy." Order ALL the reference details at left...
3.	Formaldehyde (Formol)FDA Link 01/01/1994 - "Hydrated autoclave pretreatment enhancement of prion protein immunoreactivity in formalin-fixed bovine spongiform encephalopathy-affected brain." 01/01/1994 - "The efficacy of three pretreatment techniques for the detection of prion protein (PrP) in formalin-fixed, paraffin-embedded bovine spongiform encephalopathy (BSE)--affected brain tissue were compared using automated image analysis. " 05/01/2010 - "To initially evaluate the immunoAT method, the presence or absence of prion protein (PrP(sc)) was determined in formalin-fixed and paraffin-embedded sections of the medulla oblongata of cattle which had been under active surveillance for bovine spongiform encephalopathy. " 07/01/2008 - "Collaboration was established in 2001 to evaluate a commercially available immunohistochemistry assay kit for the detection of bovine spongiform encephalopathy (BSE) disease-associated prion protein in formic acid-treated formalin-fixed samples of bovine brain. " 09/01/2010 - "Detection of PrP(Sc) in formalin-fixed, paraffin-embedded tissue by Western blot differentiates classical scrapie, Nor98 scrapie, and bovine spongiform encephalopathy." Order ALL the reference details at left...
4.	Biological Markers (Surrogate Marker)IBA 01/01/2011 - "The identification of variations in gene expression in response to bovine spongiform encephalopathy (BSE) may help to elucidate the mechanisms of neuropathology and prion replication and discover biomarkers for disease. " 08/01/2009 - "It is desirable to make the diagnosis in live cattle with bovine spongiform encephalopathy (BSE), and thus surrogate markers for the disease have been eagerly sought. " 11/01/2009 - "Cerebrospinal fluid (CSF) is considered as the most promising body fluid target for the discovery of biomarkers for early diagnosis of neurodegenerative diseases such as Creutzfeldt-Jakob disease in humans and bovine spongiform encephalopathy in cattle. " Order ALL the reference details at left...
5.	Aprotinin (Trasylol)FDA Link 06/01/1996 - "The Trasylol manufacturing process was investigated with respect to its capacity for the inactivation/removal of infectivity causing bovine spongiform encephalopathy (BSE). " 06/01/2003 - "Aprotinin improved the control of bleeding in patients undergoing surgery with cardiopulmunary bypass, but its use was halted because of the risk of bovine spongiform encephalopathy. " Order ALL the reference details at left...
6.	Sericins (Sericin)IBA 11/01/2002 - "Since BSA is obtained from bovine serum and the risk of infections such as bovine spongiform encephalopathy cannot be eradicated, sericin derived from insects could be a preferable culture medium supplement for stimulating the proliferation of mammalian cells." Order ALL the reference details at left...
7.	PrionsIBA 03/01/1993 - "Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy." 09/01/2011 - "As a result of bovine spongiform encephalopathy in Canada, specific tissues at risk of harbouring prions are not allowed to enter the food chain. " 04/01/2011 - "Atypical forms of bovine spongiform encephalopathy (BSE) may be caused by different prions from classical BSE (C-BSE). " 12/01/2010 - "Bioassays of bovine spongiform encephalopathy prions in the enzyme proceeded using knock-in mice expressing bovine prion protein. " 04/01/2010 - "Although the majority of cases occur on a sporadic basis, and rarely result from exposure to prions, such as mad cow disease, 10-15% are attributable to the presence of an autosomal dominant mutation of the prion protein gene (PRNP). " Order ALL the reference details at left...
8.	Proteins (Proteins, Gene)IBA 11/01/2008 - "To reduce the risk of the bovine spongiform encephalopathy agent being recycled to cattle through animal feed, in October 2001 Japan introduced a feed ban prohibiting the use of animal proteins in feed. " 07/25/2007 - "The feeding of ruminant proteins to ruminants is prohibited in most countries because the practice is thought to be responsible for the spread of bovine spongiform encephalopathy. " 09/01/2005 - "The use of ruminant-derived proteins in ruminant feeds has been banned in both the European Union and the United States to prevent further spread of bovine spongiform encephalopathy. " 01/01/2003 - "The risk of bovine spongiform encephalopathy propagation was drastically reduced after the European Union (EU) Health Authorities adopted restrictions involving a ban on animal-derived proteins in the diet of farm animals. " 05/01/2000 - "However, protein sources such as oilseed cakes are beyond the economic reach of most farmers, while the incidence of bovine spongiform encephalopathy in Western intensive animal production may be thought to argue against the use of animal proteins. " Order ALL the reference details at left...
9.	PrPSc ProteinsIBA 06/01/2000 - "The first objective of this study was to determine the applicability of these criteria to characterize and differentiate sheep scrapie PrP(Sc) and bovine spongiform encephalopathy (BSE) PrP(Sc). " 10/01/1997 - "An earlier study showed that, on first passage in cattle, a US scrapie agent caused an encephalopathy that was distinct from bovine spongiform encephalopathy (BSE). " 09/10/2010 - "Since 2004 cases of atypical bovine spongiform encephalopathy (BSE) in older cattle are recorded on the basis of aberrant glycoprofiles of prion protein resistant to proteolysis (PrP(res)). " 12/01/2009 - "In sheep with clinical disease, PrP(sc) was detected in the blood of 55% of scrapie agent-infected animals (n = 80) and 71% of animals with bovine spongiform encephalopathy (n = 7). " 01/01/2009 - "To investigate the relative involvement of the olfactory region in classical bovine spongiform encephalopathy (BSE), immunohistochemical labeling of prion protein scrapie (PrP(Sc)) was scored in the brainstem, frontal cerebral cortex, and olfactory bulb of cattle with natural and experimental clinical cases of BSE in Great Britain. " Order ALL the reference details at left...
10.	Haemophilus influenzae type b-polysaccharide vaccine-diphtheria toxoid conjugateIBA 06/01/2009 - "This study characterizes the traumatic brain lesions associated with the I/C injection of the bovine spongiform encephalopathy agent in sheep, assesses the relevance of such lesions in the outcome of clinical disease and provides insight into the mechanisms of PrP(d) conversion and amplification following I/C challenge. " 06/01/2009 - "To determine the nature of subcellular changes and their association with PrP(d) we perfusion-fixed brains of eight bovine spongiform encephalopathy (BSE)-affected cows and three control cattle for immunogold electron microscopy at two different neuroanatomical sites. " 02/01/2006 - "Immunohistochemical epitope mapping of abnormal PrP accumulations (PrP(d)) in brain can help in characterizing sheep TSE sources or strains and in identifying potential bovine spongiform encephalopathy (BSE) infections of sheep. " 03/01/2005 - "It has previously been reported that disease-associated prion protein (PrP(d)) derived from natural scrapie and from sheep infected experimentally with bovine spongiform encephalopathy (BSE) differed in respect of their immunohistochemical and immunoblotting properties. " 05/01/2003 - "We have now extended those studies to investigate the effect of different PrP antibodies on the PrP(d) profile of scrapie- and bovine spongiform encephalopathy (BSE)-affected sheep. " Order ALL the reference details at left...

Therapies and Procedures

1.	Blood Transfusion (Blood Transfusions) 12/01/2008 - "The emergence of variant Creutzfeld-Jakob disease, following on from the bovine spongiform encephalopathy (BSE) epidemic, led to concerns about the potential risk of iatrogenic transmission of disease by blood transfusion and the introduction of costly control measures to protect blood supplies. " 05/01/2004 - "Bovine spongiform encephalopathy, variant CJD, and blood transfusion: beefer madness?" 06/01/2003 - "The risks of transmission of the so called "mad cow disease" are now under control but concerns still persist about potential secondary transmissions, notably via blood transfusion. " 09/01/1996 - "A matter for debate: the risk of bovine spongiform encephalopathy to humans posed by blood transfusion in the UK." 09/01/2009 - "The epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom, its related occurrence of a new type of Creutzfeld-Jacob disease and proven cases of this type of the disease transmitted by blood transfusion initiated intensive studies to develop a inexpensive, prompt, and sensitive method for the early lifetime diagnosis of prion diseases. " Order ALL the reference details at left...
2.	Surgical Instruments (Clip) 05/01/2004 - "For the assessment of risk scenarios originating from these findings (e.g., alimentary transmission of pathogens associated with bovine spongiform encephalopathy [BSE] and chronic wasting disease [CWD] via tainted beef and game or iatrogenic dissemination of CJD agent through contaminated surgical instruments) more detailed information about the time course of PrP(Sc) accumulation in muscles at preclinical and clinical stages of incubation is needed. " 04/01/1999 - "It lends itself to a systematic study of the conditions required to effectively inactivate CJD, bovine spongiform encephalopathy, and scrapie agent adsorbed to stainless steel surfaces such as those of surgical instruments." Order ALL the reference details at left...
3.	Prostheses and Implants (Prosthesis) 05/01/2000 - "Production of the Bioflo pericardial prosthesis used in this trial was discontinued due to fears related to bovine spongiform encephalopathy. " Order ALL the reference details at left...
4.	Transplants (Transplant) 06/01/2001 - "Analysis of the risk of transmitting bovine spongiform encephalopathy through bone grafts derived from bovine bone." 09/01/1999 - "Risk assessment of bovine spongiform encephalopathy transmission through bone graft material derived from bovine bone used for dental applications." 01/01/1996 - "As for bovine bone grafts in recent times attention has been paid to the diffusion in various countries of the European Economic Community of "bovine spongiform encephalopathy"; this pertains to the same group of encephalopathies as Creutzfeldt-Jakob disease which strikes humans and has a lethal outcome. " 11/01/2000 - "Even before bovine spongiform encephalopathy and nvCJD were linked, the recognition that iatrogenic Creutzfeldt-Jakob disease (CJD) had been acquired from administration of cadaveric human growth and gonadotropic hormones and from corneal and dura mater transplants prompted medical vigilance. " 05/01/1999 - "Despite these advances, we have seen in recent years 3 potentially preventable outbreaks of disease: iatrogenic Creutzfeldt-Jakob disease due to contaminated pituitary hormone extracts and dura mater grafts, and a new variant of the disease (Will-Ironside syndrome) linked to consumption of beef contaminated by the agent of bovine spongiform encephalopathy, underscoring the disparity between fundamental knowledge and its practical application." Order ALL the reference details at left...
5.	Heterologous Transplantation (Xenotransplantation) 01/01/2000 - "Potential applications in both the bovine and porcine species are described with particular emphasis on the generation of cattle resistant to bovine spongiform encephalopathy (BSE) and pigs that can be of use in xenotransplantation." 05/01/1998 - "The concern that xenotransplantation carries the potential for a new epidemic has been highlighted by recent experiences with both bovine spongiform encephalopathy and human immunodeficiency diseases. " Order ALL the reference details at left...

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