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Occurrence of lysoganglioside lyso-GM2 (II3-Neu5Ac-gangliotriaosylsphingosine) in GM2 gangliosidosis brain
Authors:S Neuenhofer  E Conzelmann  G Schwarzmann  H Egge  K Sandhoff
Abstract:Lysoganglioside lyso-GM2 (sialylgangliotriaosylsphingosine) was detected in a brain sample of a patient with variant B of infantile GM2 gangliosidosis (Tay-Sachs disease) at a concentration of approx. 15 nmol/g wet tissue. Neither lysoganglioside lyso-GM2 nor the corresponding GM1 derivative could be detected in normal brain.
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