Abstract
A golden retriever dog is described with total hexosaminidase deficiency and raised GM2-ganglioside in CSF. The animal represents a model for human Sandhoff disease.
MeSH terms
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Animals
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Behavior, Animal
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Dog Diseases / genetics*
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Dogs
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Lysosomes / enzymology
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Male
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Nervous System Diseases / etiology
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Nervous System Diseases / physiopathology
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Sandhoff Disease / genetics
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Sandhoff Disease / psychology
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Sandhoff Disease / veterinary*
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beta-N-Acetylhexosaminidases / cerebrospinal fluid
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beta-N-Acetylhexosaminidases / deficiency
Substances
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beta-N-Acetylhexosaminidases