Incidence and characterization of polyglucosan bodies in the cerebella of montserrat orioles ( <i>Icterus oberi</i> )

Polyglucosan bodies are accumulations of insoluble glucose polymers and proteins that form intracytoplasmic inclusions in the brain, large numbers of which can be indicative of neurodegenerative diseases such as Lafora disease. Montserrat orioles (<i>Icterus oberi</i>) are an icterid passerine endemic to Montserrat with conservation populations maintained in captivity abroad. We demonstrate that polyglucosan bodies are unusually abundant in the cerebellar molecular and Purkinje cell layers and cerebellar peduncles of captive-bred and wild-caught Montserrat orioles. The bodies are periodic acid-Schiff positive and diastase resistant and label with concanavalin A and for ubiquitin, consistent with those seen in humans. We found no association of the polyglucosan bodies with concurrent neurological lesions or clinical signs, nor with <i>EPM2</i>A and <i>EPM2B</i> gene mutations associated with Lafora disease. We conclude that an abundance of cerebellar polyglucosan bodies may be a normal finding in aged Montserrat orioles and not a threat to the captive breeding population.

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Publication Details

Journal
Veterinary Pathology
Published
2024-08-15
DOI
https://doi.org/10.1177/03009858241270000
Citations
2
Primary Topic
Glycogen Storage Diseases and Myoclonus
Type
article
Field-Weighted Citation Impact
0.94
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article

Incidence and characterization of polyglucosan bodies in the cerebella of montserrat orioles ( Icterus oberi )

Adrian L. Smith, Simon Spiro, Mark F. Stidworthy, M. de A. Pereira et al.
2 citations
Veterinary Pathology
Glycogen Storage Diseases and Myoclonus
0.94
article

Incidence and characterization of polyglucosan bodies in the cerebella of montserrat orioles ( Icterus oberi )

Adrian L. Smith, Simon Spiro, Mark F. Stidworthy, M. de A. Pereira, Emma Nye, Alejandro Núñez, Alberto Rodríguez Barbón, David J. Everest, Daniela Denk, Kieran A. Bates, Steven R. Fiddaman, Zane Jaunmuktane, Ethan Wrigglesworth, A Theodoulou, Yan Liu
article en
2 citations

Abstract

Polyglucosan bodies are accumulations of insoluble glucose polymers and proteins that form intracytoplasmic inclusions in the brain, large numbers of which can be indicative of neurodegenerative diseases such as Lafora disease. Montserrat orioles (<i>Icterus oberi</i>) are an icterid passerine endemic to Montserrat with conservation populations maintained in captivity abroad. We demonstrate that polyglucosan bodies are unusually abundant in the cerebellar molecular and Purkinje cell layers and cerebellar peduncles of captive-bred and wild-caught Montserrat orioles. The bodies are periodic acid-Schiff positive and diastase resistant and label with concanavalin A and for ubiquitin, consistent with those seen in humans. We found no association of the polyglucosan bodies with concurrent neurological lesions or clinical signs, nor with <i>EPM2</i>A and <i>EPM2B</i> gene mutations associated with Lafora disease. We conclude that an abundance of cerebellar polyglucosan bodies may be a normal finding in aged Montserrat orioles and not a threat to the captive breeding population.

Veterinary PathologyVol. 62(4)
SeaWorld Entertainment (US), Zoological Society of London (GB), Durrell Wildlife Conservation Trust (JE), University College London Hospitals NHS Foundation Trust (GB), University of Nottingham (GB), Queen Mary University of London (GB), Animal and Plant Health Agency (GB), Chester Zoo (GB), The Francis Crick Institute (GB), University of Oxford (GB), Wildlife Vets International (GB), National Hospital for Neurology and Neurosurgery (GB), University College London (GB), Imperial College London (GB)
Life in Land
Openalex Percentile: Top 24%
Glycogen Storage Diseases and Myoclonus
0.94
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