title: A Naturally Occurring Bovine Tauopathy Is Geographically Widespread in the UK.
abstract: Many human neurodegenerative diseases are associated with hyperphosphorylation and widespread intra-neuronal and glial associated aggregation of the microtubule associated protein tau. In contrast, animal tauopathies are not reported with only senescent animals showing inconspicuous tau labelling of fine processes albeit significant tau aggregation may occur in some experimental animal disease. Since 1986, an idiopathic neurological condition of adult cattle has been recognised in the UK as a sub-set of cattle slaughtered as suspect bovine spongiform encephalopathy cases. This disorder is characterised by brainstem neuronal chromatolysis and degeneration with variable hippocampal sclerosis and spongiform change. Selected cases of idiopathic brainstem neuronal chromatolysis (IBNC) were identified from archive material and characterised using antibodies specific to several tau hyperphosphorylation sites or different isoforms of the tau microtubule binding region. Labelling was also carried out for alpha synuclein, ubiquitin, TDP43, Aβ 1-42, Aβ 1-40. Widespread tau labelling was identified in all IBNC brains examined and with each of seven tau antibodies recognising different hyperphosphorylated sites. Labelling with each antibody was associated with dendrites, neuronal perikarya and glia. Thus IBNC is a sporadic, progressive neurological disease predominantly affecting aged cattle that occurs throughout the UK and is associated with hyperphosphorylation of tau, a rare example of a naturally-occurring tauopathy in a non-primate species. Secondary accumulation of alpha synuclein and ubiquitin was also present. The neuropathology does not precisely correspond with any human tauopathy. The cause of IBNC remains undetermined but environmental factors and exposure to agrochemicals needs to be considered in future aetiological investigations.
authors.affiliation: Animal and Plant Health Agency, Lasswade Veterinary Laboratory, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, Scotland.
authors.forename: Martin
authors.initials: M
authors.lastname: Jeffrey
authors.affiliation: Laboratory of Bacterial and TSE-agents, Food and Drug Administration, Rockville, Maryland, United States of America.
authors.forename: Pedro
authors.initials: P
authors.lastname: Piccardo
authors.affiliation: National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, United Kingdom.
authors.forename: Diane L
authors.initials: DL
authors.lastname: Ritchie
authors.affiliation: National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, United Kingdom.
authors.forename: James W
authors.initials: JW
authors.lastname: Ironside
authors.affiliation: National CJD Research & Surveillance Unit, Centre for Clinical Brain Sciences, School of Clinical Sciences, University of Edinburgh, Western General Hospital, Crewe Road, Edinburgh, United Kingdom.
authors.forename: Alison J E
authors.initials: AJ
authors.lastname: Green
authors.affiliation: Animal and Plant Health Agency, Lasswade Veterinary Laboratory, Pentlands Science Park, Bush Loan, Penicuik, Midlothian, Scotland.
authors.forename: Gillian
authors.initials: G
authors.lastname: McGovern
mesh_terms: D000818:Animals; D001921:Brain / Q000378:metabolism / Q000473:pathology; D002417:Cattle; D002418:Cattle Diseases / Q000453:epidemiology* / Q000209:etiology*; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology / Q000209:etiology; D006624:Hippocampus / Q000378:metabolism / Q000473:pathology; D006801:Humans; D015994:Incidence; D009457:Neuroglia / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D020033:Protein Isoforms; D024801:Tauopathies / Q000662:veterinary*; D006113:United Kingdom / Q000453:epidemiology; D051844:alpha-Synuclein / Q000378:metabolism; D016875:tau Proteins / Q000378:metabolism
chemical_list: D020033:Protein Isoforms; D051844:alpha-Synuclein; D016875:tau Proteins