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| Product Name | β-Galactosidase1 (Galactosidase, beta 1, GLB1) |
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| Description | Purity ~95% (SDS-PAGE). GLB1 is a lysosomal Bgalactosidase that hydrolyzes the terminal Bgalactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1gangliosidosis and Morquio B syndrome (also known as mucopolysaccharidosis IVB) (1, 2, 3). In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0 ±3% of normal) of lysosomal Bgalactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate (4). More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in th |
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| Size | 20ug |
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| Concentration | n/a |
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| Applications | n/a |
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| Other Names | n/a |
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| Gene, Accession, CAS # | Accession: AAA51819 |
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| Catalog # | G1041-47J |
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| Order / More Info | β-Galactosidase1 (Galactosidase, beta 1, GLB1) from UNITED STATES BIOLOGICAL |
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| Product Specific References | n/a |
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