Lysosomal storage of oligosaccharide and glycosphingolipid in imino sugar treated cells.

Sandhoff and Tay-Sachs disease are autosomal recessive GM2 gangliosidoses where a deficiency of lysosomal beta-hexosaminidase results in storage of glycoconjugates. Imino sugar (2-acetamido-1,4-imino-1,2,4-trideoxy-L-arabinitol) inhibition of beta-hexosaminidase in murine RAW264.7 macrophage-like ce...

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Bibliografiska uppgifter
Huvudupphovsmän: Boomkamp, S, Rountree, J, Neville, D, Dwek, R, Fleet, G, Butters, T
Materialtyp: Journal article
Språk:English
Publicerad: 2010