ganglioside gm2造句

• Gangliosides are lipids, components of cellular membranes, and the ganglioside GM2, implicated in Tay-Sachs disease, is especially common in the nervous tissue of the brain.
• The protein encoded by this gene is a small glycolipid transport protein which acts as a substrate specific co-factor for the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
• This protein binds molecules of ganglioside GM2, extracts them from membranes, and presents them to beta-hexosaminidase A for cleavage of N-acetyl-D-galactosamine and conversion to GM3.
• Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.
• "' Sandhoff disease "', also known as "'Sandhoff-Jatzkewitz disease "', "'variant 0 of GM2-Gangliosidosis "'or "'Hexosaminidase A and B deficiency "', is a lysosomal genetic, lipid storage disorder caused by the inherited deficiency to create functional beta-hexosaminidases A and B . These catabolic enzymes are needed to degrade the neuronal membrane components, ganglioside GM2, its derivative GA2, the glycolipid globoside in visceral tissues, The rare autosomal recessive neurodegenerative disorder is clinically almost indistinguishable from Tay-Sachs disease, another genetic disorder that disrupts beta-hexosaminidases A and S . There are three subsets of Sandhoff disease based on when first symptoms appear : classic infantile, juvenile and adult late onset.