globoside การใช้

• The deficiency of ?-galactosidase A causes Fabry's disease, an inherited metabolic disease characterized by the accumulation of the globoside globotriaosylceramide.
• In humans the P antigen ( also known as globoside ) is the cellular receptor for parvovirus B19 virus that causes Erythema infectiosum ( fifth disease ) in children.
• He also made significant contributions to the understanding of parvovirus B19 infection in hematopoietic cells; he identified the erythrocyte antigen P ( globoside ) as the cell receptor for parvovirus B19 and that P antigen deficiency confers resistance to parvovirus B19 infection.
• In humans, the P antigen ( also known as globoside ), one of the many cellular receptors that contribute to a person's blood type, is the cellular receptor for parvovirus B19 virus that causes erythema infectiosum ( fifth disease ) in children.
• Besides the neuronal storage of GM2, the storage of GA2 was much more pronounced, and different from all cases of Tay-Sachs disease studied so far, globoside accumulated in the visceral organs and, most importantly, hexosaminidase activity was almost completely absent.
• The disease causing catabolic enzyme deficiency of hexosaminidases was demonstrated with four different substrates ( p nitrophenyl-?-D-N-acetylglucosaminide, p-nitrophenyl-?-D-N-acetylgalactosaminide, glycolipid [ 3H ] GA2 and [ 3H ] globoside ) in four different organs and published in 1968.
• "' 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.