Occurrence of lysoganglioside lyso-GM2 (II3-Neu5Ac-gangliotriaosylsphingosine) in GM2 gangliosidosis brain.
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.
S
S
Neuenhofer
E
E
Conzelmann
G
G
Schwarzmann
H
H
Egge
K
K
Sandhoff
D001923:Brain Chemistry; D005678:G(M2) Ganglioside / Q000031:analogs & derivatives / Q000032:analysis*; D005732:Gangliosides / Q000032:analysis*; D006801:Humans; D013058:Mass Spectrometry; D012016:Reference Values; D013661:Tay-Sachs Disease / Q000378:metabolism*
D005732:Gangliosides; C048941:lysoganglioside G(M2); D005678:G(M2) Ganglioside