{"id":23941,"date":"2025-04-23T13:38:30","date_gmt":"2025-04-23T13:38:30","guid":{"rendered":"https:\/\/labgenvet.ca\/?page_id=23941"},"modified":"2025-04-23T13:41:05","modified_gmt":"2025-04-23T13:41:05","slug":"spongiform-encephalopathy-bse-susceptibility","status":"publish","type":"page","link":"https:\/\/labgenvet.ca\/en\/spongiform-encephalopathy-bse-susceptibility\/","title":{"rendered":"Spongiform encephalopathy (BSE), susceptibility"},"content":{"rendered":"

Spongiform encephalopathy (BSE), susceptibility<\/strong><\/h1>\n

 <\/p>\n

Gene<\/strong>:\u00a0PRNP<\/p>\n

Transmission<\/strong>: Unknown<\/p>\n

Mutation<\/strong>: Substitution, PRNP gene; c.631 G>A, p.(Glu211Lys), chromosome 13.<\/p>\n

Breed<\/strong>: Cattle, ruminants, horse, wild mammals, humans.<\/p>\n

Medical system:<\/strong>\u00a0Neurological<\/p>\n

Age of onset of symptoms<\/strong>: Long incubation time (2.5 \u2013 8 years).<\/p>\n

Spongiform encephalopathy, also known as Bovine Spongiform Encephalitis (BSE) or Mad Cow disease, is a type of fatal neurodegenerative disease seen in cattle.\u00a0 BSE is similar to scrapies seen in sheep and goats, chronic wasting disease seen in deer, and Creutzfeld-Jakob disease (CJD) seen in humans.\u00a0 These diseases are known as prion diseases and are caused by a misfolded natural protein, PrP, coded for by the PRNP gene.\u00a0 The natural protein is a widely expressed cell membrane glycoprotein whose function is unknown.\u00a0 Misfolding of the PrP protein by inappropriate glycosylation and disulfide bonding results in a scrapie-Prp protein that can \u201crecruit\u201d normal PrP proteins to misfold, making the scrapie-PrP protein an infectious agent.\u00a0 Notably, the normal PrP and the scrapie-Prp proteins can have the same amino acid primary structure and be different only in secondary conformation.\u00a0 The production of scrapie-PrP proteins results in amyloid accumulation in brain tissues and the neurological symptoms associated with the disease.\u00a0 These symptoms include progressive signs of ataxia, tremors, incoordination, weight loss, decreased production, aggression and eventually recumbency and death.\u00a0 There is no treatment.\u00a0 Diagnosis is post-mortem.<\/p>\n

Two types of BSE are described in cattle, the classical type and the atypical type.\u00a0 Classical BSE occurred in the United Kingdom during the 1980s and 1990s, as a result of cattle consuming prion contaminated feed.\u00a0 Both horizontal and vertical transmission in cattle was seen.\u00a0 Notably, classical BSE was transmissible to humans who had consumed contaminated bovine products, resulting in a variation of Creutzfeld-Jakob disease. The atypical type of BSE appears sporadically in cattle at an estimated frequency of 1 case in 1 million animals.\u00a0 Familial cases of CJD are seen in humans and are often associated with a germline mutation (E210K) in the PrP protein sequence.\u00a0 The equivalent mutation in the bovine PrP protein sequence (E211K) has been identified in somatic tissues from one animal diagnosed with atypical BSE and from several animals not exhibiting symptoms of BSE. \u00a0The susceptibility or resistance to BSE conferred by this mutation requires further investigation.<\/p>\n

Bovine spongiform encephalopathy has been known to cause disease in humans.\u00a0 Appropriate measures and reporting should be exercised.<\/p>\n

\u00a0<\/strong><\/p>\n

References:<\/strong><\/p>\n

OMIA link: [0944-9913<\/a>]<\/p>\n

Konold T, Rajanayagam B, Meldrum K. (2025<\/strong>) Atypical BSE in cattle.\u00a0Vet Rec\u00a0196:275-276.\u00a0 [40152492<\/a>]<\/p>\n

Lestari TD, Khairullah AR, Utama S, et al. (2025<\/strong>) Bovine spongiform encephalopathy: A review of current knowledge and challenges.\u00a0Open Vet J\u00a015:54-68.\u00a0 [40092198<\/a>]<\/p>\n

Kim YC, Park KJ, Hwang JY, et al. (2022<\/strong>) In-depth examination of PrP Sc in Holstein cattle carrying the E211K somatic mutation of the bovine prion protein gene (PRNP).\u00a0Transbound Emerg Dis\u00a069:e356-e361.\u00a0 [pm\/34470082<\/a>]<\/p>\n

Won S-Y, Kim Y-C, Jeong B-H. (2020) First report of the potential bovine spongiform encephalopathy (BSE)-related somatic mutation E211K of the prion protein gene (PRNP) in cattle.\u00a0 Int J Mol Sci 21(12):4246.\u00a0 [pm\/32549191<\/a>]<\/p>\n

Brunelle BW, Greenlee JJ, Seabury CM, et al. (2008<\/strong>) Frequencies of polymorphisms associated with BSE resistance differ significantly between Bos taurus, Bos indicus, and composite cattle.\u00a0BMC Vet Res\u00a04:36.\u00a0 [pm\/18808703<\/a>]<\/p>\n

Richt JA, Hall, SM. (2008<\/strong>) BSE case associated with prion protein gene mutation.\u00a0PLoS Pathog\u00a04:e1000156.\u00a0 [pm\/18787697<\/a>]<\/p>\n

 <\/p>\n

Contributed by: Aur\u00e9lie Moreau and Sofia Pinero-Tabah, Class of 2029, Facult\u00e9 de m\u00e9decine v\u00e9t\u00e9rinaire, Universit\u00e9 de Montr\u00e9al.\u00a0 (Translation DWS)<\/p>\n","protected":false},"excerpt":{"rendered":"

Spongiform encephalopathy (BSE), susceptibility   Gene:\u00a0PRNP Transmission: Unknown Mutation: Substitution, PRNP gene; c.631 G>A, p.(Glu211Lys), chromosome 13. Breed: Cattle, ruminants, horse, wild mammals, humans. Medical system:\u00a0Neurological Age of onset of symptoms: Long incubation time (2.5 \u2013 8 years). Spongiform encephalopathy, also known as Bovine Spongiform Encephalitis (BSE) or Mad Cow disease, is a type of…<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"footnotes":""},"class_list":["post-23941","page","type-page","status-publish","hentry","description-off"],"acf":[],"aioseo_notices":[],"_links":{"self":[{"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/pages\/23941","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/comments?post=23941"}],"version-history":[{"count":3,"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/pages\/23941\/revisions"}],"predecessor-version":[{"id":23944,"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/pages\/23941\/revisions\/23944"}],"wp:attachment":[{"href":"https:\/\/labgenvet.ca\/en\/wp-json\/wp\/v2\/media?parent=23941"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}