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  • Sandhoff¡¯s disease
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  • sandhoff disease
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SD Sandhoff disease; senile dementia; septal defect; serologically defined; serologically detectable; s...
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Sandhoff, K <person> Contemporary German biochemist.
See: Sandhoff's disease.
(05 Mar 2000)
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sandhoff disease A variant of tay-sachs disease. It is caused by a deficiency of hexosaminidases a & b inherited as an autosomal recessive trait, leading to accumulation of g(m2) ganglioside and the sphingolipid globoside in neurons. The diseases manifests clinically as psychomotor retardation and deterioration, blindness, cherry red spot in the macula, hepatosplenomegaly, and death in 1-3 years.
(12 Dec 1998)
Sandhoff's disease An infantile form of GM2 gangliosidosis characterised by a defect in the production of hexosaminidases A and B; it resembles Tay-Sachs disease, but occurs predominantly (if not entirely) in non-Jewish children; accumulation of glucoside and ganglioside Gm2.
(05 Mar 2000)
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