Abstract
BackgroundGM1 and GM2 gangliosidoses are progressive neurodegenerative lysosomal storage diseases resulting from the excessive accumulation of GM1 and GM2 gangliosides in the lysosomes, respectively. The diagnosis of gangliosidosis is carried out based on comprehensive findings using various types of specimens for histological, ultrastructural, biochemical and genetic analyses. Therefore, the partial absence or lack of specimens might have resulted in many undiagnosed cases. The aim of the present study was to establish immunohistochemical and immunofluorescent techniques for the auxiliary diagnosis of canine and feline gangliosidoses, using paraffin-embedded brain specimens stored for a long period.ResultsUsing hematoxylin and eosin staining, cytoplasmic accumulation of pale to eosinophilic granular materials in swollen neurons was observed in animals previously diagnosed with GM1 or GM2 gangliosidosis. The immunohistochemical and immunofluorescent techniques developed in this study clearly demonstrated the accumulated material to be either GM1 or GM2 ganglioside.ConclusionsImmunohistochemical and immunofluorescent techniques using stored paraffin-embedded brain specimens are useful for the retrospective diagnosis of GM1 and GM2 gangliosidoses in dogs and cats.
Highlights
GM1 and GM2 gangliosidoses are progressive neurodegenerative lysosomal storage diseases resulting from the excessive accumulation of GM1 and GM2 gangliosides in the lysosomes, respectively
Using the hematoxylin and eosin (HE) stain, cytoplasmic accumulation of pale to eosinophilic granular materials in balloon-swollen neurons was observed in the cerebral cortex samples of dogs and cats previously diagnosed with GM1 or GM2 gangliosidosis (Fig. 1a–d), whereas there was no such abnormal change observed in the samples of the control animals (Fig. 1e and f )
In animals with GM2 gangliosidosis, the accumulated cytoplasmic materials were very weakly positively stained in a portion of the cells of the affected cat (Fig. 2c and d)
Summary
GM1 and GM2 gangliosidoses are progressive neurodegenerative lysosomal storage diseases resulting from the excessive accumulation of GM1 and GM2 gangliosides in the lysosomes, respectively. GM1 and GM2 gangliosidoses are progressive neurodegenerative lysosomal storage diseases resulting mainly from the excessive accumulation of GM1 and GM2 gangliosides in the lysosomes, respectively [1]. Occurring GM2 gangliosidosis has been reported in dogs, including German Shorthair Pointers [24], Japanese Spaniels (Chins) [25, 26], a Golden Retriever [27], Toy Poodles [28], and mixed-breed dogs [29, 30], and in cats, including Korat [31], European Burmese [32], and several families of domestic cats [33,34,35]. GM2 gangliosidosis has been reported in Yorkshire pigs [36], Jacob sheep [37], a rabbit [38], Muntjak deer [39], and American flamingos [40]
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