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...

وصف كامل

التفاصيل البيبلوغرافية
المؤلفون الرئيسيون: Boomkamp, S, Rountree, J, Neville, D, Dwek, R, Fleet, G, Butters, T
التنسيق: Journal article
اللغة:English
منشور في: 2010