| Gangliosidosis in emus (Dromaius novaehollandiae). | |
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MedLine Citation:
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PMID: 7677649 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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A 6-month-old female emu (Dromaius novaehollandiae) died following acute central nervous system signs. Hematoxylin-and-eosin-stained sections revealed that neurons of the brain were distended with nonstaining 1-to-2-microns vacuoles. Ultrastructural examination of the affected neurons revealed numerous membranous cytoplasmic bodies (MCBs) similar in appearance to the MCBs seen in mammalian gangliosidoses. A full sibling of this emu was donated for study. This 7-month-old female emu was stunted compared with hatchmates. Neurologic examination revealed hypermetric gait, persistent head tremor, and mild ataxia. No gross lesions were evident at postmortem. Histopathologic and electron microscopic findings were similar to those in the index case in that swollen, pale neurons were present in the cerebrum, pons, medulla, cerebellum, spinal cord, spinal ganglia, autonomic ganglia, myenteric plexus, and ganglion cell layer of the retina. Analysis of brain gangliosides of the affected 7-month-old emu revealed 14- and 25-fold increases of GM1 and GM3 gangliosides, respectively, compared with control emus. The total brain ganglioside sialic acids were, on a wet weight basis, 519 micrograms/g (control A), 658 micrograms/g (control B), and 1800 micrograms/g (affected emu). The familial association seen with this condition suggests that emus are affected by an inherited disorder similar to mammalian gangliosidoses. |
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Authors:
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A J Bermudez; G C Johnson; M T Vanier; M Schröder; K Suzuki; P L Stogsdill; G S Johnson; D O'Brien; C P Moore; W W Fry |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Avian diseases Volume: 39 ISSN: 0005-2086 ISO Abbreviation: Avian Dis. Publication Date: 1995 Apr-Jun |
Date Detail:
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Created Date: 1995-10-19 Completed Date: 1995-10-19 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0370617 Medline TA: Avian Dis Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 292-303 Citation Subset: IM |
Affiliation:
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College of Veterinary Medicine, University of Missouri-Columbia 65211, USA. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Animals Bird Diseases / enzymology, pathology, physiopathology* Brain / enzymology, ultrastructure Chromatography, Thin Layer / veterinary Female Gangliosides / metabolism Gangliosidoses / enzymology, pathology, physiopathology, veterinary* Microscopy, Electron / veterinary |
| Grant Support | |
ID/Acronym/Agency:
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P30-HD03110/HD/NICHD NIH HHS; R01-NS 242889/NS/NINDS NIH HHS; R01-NS 28977/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Gangliosides |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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