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...
| Main Authors: | , , , , , |
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| Format: | Journal article |
| Language: | English |
| Published: |
2010
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