title: Neuronal intranuclear inclusion disease in a horse.
abstract: Neuronal intranuclear inclusion disease (NIID) is reported in a 16-year-old Pure Spanish breed female horse suffering from progressive ataxia and motor deficiencies. The neuropathological study revealed NIIs throughout the central nervous system, although mainly in the brain stem and spinal cord. This distribution did not correlate with neuron loss, which was marked in the hippocampus and moderate in the neocortex, particularly in the occipital cortex. As in humans, NIIs in the horse were hyaline autofluorescent inclusions composed of non-membrane-bound aggregates of filaments and fine granules. NIIs were stained with anti-ubiquitin and anti-clusterin antibodies. In addition, NIIs were stained with antibodies raised against subunits of the 19S and PA28, but not of the 20S, components of the proteasome. These observations indicate similarities between NIID in humans and horses, and suggest that clusterin and abnormal ubiquitin-proteasomal expression participate in NII formation.
authors.affiliation: Animal Tissue Bank of Catalunya, Department of Animal Medicine and Surgery, Universitat Autònoma de Barcelona, 08193, Bellaterra (Cerdanyola del Vallès) Barcelona, Spain. marti.pumarola@uab.es
authors.forename: M
authors.initials: M
authors.lastname: Pumarola
authors.forename: E
authors.initials: E
authors.lastname: Vidal
authors.forename: J M
authors.initials: JM
authors.lastname: Trens
authors.forename: A
authors.initials: A
authors.lastname: Serafín
authors.forename: M
authors.initials: M
authors.lastname: Marquez
authors.forename: I
authors.initials: I
authors.lastname: Ferrer
mesh_terms: D000818:Animals; D001921:Brain / Q000473:pathology* / Q000648:ultrastructure; D051152:Clusterin / Q000378:metabolism; D005260:Female; D006736:Horses; D006801:Humans; D007150:Immunohistochemistry; D045586:Intranuclear Inclusion Bodies / Q000473:pathology*; D046529:Microscopy, Electron, Transmission; D009124:Muscle Proteins / Q000378:metabolism; D019636:Neurodegenerative Diseases / Q000473:pathology* / Q000662:veterinary*; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism; D013116:Spinal Cord / Q000473:pathology / Q000648:ultrastructure; D025801:Ubiquitin / Q000378:metabolism
chemical_list: D051152:Clusterin; D009124:Muscle Proteins; D025801:Ubiquitin; C486207:PSME2 protein, human; D046988:Proteasome Endopeptidase Complex