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Acta Neuropathologica
Published in: Acta Neuropathologica 2/2005

01-08-2005 | Case Report

Neuronal intranuclear inclusion disease in a horse

Authors: M. Pumarola, E. Vidal, J. M. Trens, A. Serafín, M. Marquez, I. Ferrer

Published in: Acta Neuropathologica | Issue 2/2005

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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.
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Metadata
Title
Neuronal intranuclear inclusion disease in a horse
Authors
M. Pumarola
E. Vidal
J. M. Trens
A. Serafín
M. Marquez
I. Ferrer
Publication date
01-08-2005
Publisher
Springer-Verlag
Published in
Acta Neuropathologica / Issue 2/2005
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI
https://doi.org/10.1007/s00401-005-1033-5

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