کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2437556 1553765 2013 5 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
GM2 Gangliosidosis in an Adult Pet Rabbit
موضوعات مرتبط
علوم زیستی و بیوفناوری علوم کشاورزی و بیولوژیک علوم دامی و جانورشناسی
پیش نمایش صفحه اول مقاله
GM2 Gangliosidosis in an Adult Pet Rabbit
چکیده انگلیسی

SummaryA 1.5-year-old neutered male rabbit was presented with chronic nasal discharge and ataxia. Rapid progression of neurological signs was noted subsequent to general anaesthesia and the rabbit was humanely destroyed due to the poor prognosis. At necropsy examination there were no gross changes affecting the brain or spinal cord. Microscopical examination revealed that the perikarya of numerous neurons in the brain and spinal cord were distended by the intracytoplasmic accumulation of pale, finely granular to vacuolar material. Transmission electron microscopy showed this to be composed of concentric membranous cytoplasmic bodies. Thin layer chromatography revealed elevation of GM2 ganglioside in the brain of this rabbit compared with that of an unaffected control rabbit. Enzymatically, there was markedly reduced activity of tissue β-hexosaminidase A in brain and liver tissue from the rabbit. This was a result of an almost complete absence of the enzymatic activity of the α-subunit of that enzyme. These findings are consistent with sphingolipidosis comparable with human GM2 gangliosidosis variant B1.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Journal of Comparative Pathology - Volume 148, Issues 2–3, February–April 2013, Pages 243–247
نویسندگان
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