Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system
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Synthesis of novel NBD-GM1 and NBD-GM2 for the transfer activity of GM2-activator protein by a FRET-based assay system.
The ganglioside-activator protein is an essential cofactor for the lysosomal degradation of ganglioside GM2 (GM2) by beta-hexosaminidase A. It mediates the interaction between the water-soluble exohydrolase and its membrane-embedded glycolipid substrate at the lipid-water interphase. Mutations in the gene encoding this glycoprotein result in a fatal neurological storage disorder, the AB variant...
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A protein activator (GMz-activator) specific for stimulating the hydrolysis of G M ~ ganglioside (GalNAcb1 4GalC3 + 2aNeuAc]/?l-+ 4Glcb1-+ 1’Cer) by &hexosaminidase A has been purified over 1OS-fold with a high yield from human liver. The purification procedure includes the adjustment of the pH of liver extract to pH 4.3, followed by ammonium sulfate precipitation, Sephadex G-200 filtration, an...
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15 صفحه اولTwo polymorphisms for the human GM2 activator protein gene.
Source/Description: A 300 bp HincII/EcoRI fragment of the human G^ activation protein cDNA (1) was subcloned into Bluescript vector and used as a probe. Polymorphism: Mspl identifies a two allele polymorphism with bands at either 3.4 kb (Al) or 2.9 kb (A2) and invariant bands at 1.0 kb, 1.8 kb and 4.0 kb. Rsal identifies a two allele polymorphism with a band at either 4.1 kb (Bl) or 3.4 kb (B2)...
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GM2 gangliosidoses are autosomal recessive lysosomal storage diseases (LSDs) caused by mutations in the HEXA, HEXB and GM2A genes, which encode the human lysosomal β-hexosaminidase (Hex) α- and β-subunits, and GM2 activator protein (GM2A), respectively. These diseases are associated with excessive accumulation of GM2 ganglioside (GM2) in the brains of patients with neurological symptoms. Here w...
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ژورنال
عنوان ژورنال: Glycobiology
سال: 2005
ISSN: 1460-2423,0959-6658
DOI: 10.1093/glycob/cwj018