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Product Name
Human GLB1/ELNR1 (His tag) recombinant protein
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Description
β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases.
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Protein name
galactosidase beta 1(GLB1)
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Protein short names
ELNR1
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Uniprot ID
P16278
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Source/Expression Host
Human Cells
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Expression Plasmid/cDNA
A DNA sequence encoding the Human GLB1/ELNR1 Leu24-Val677 is expressed with a 6His tag at the C-terminus.
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Protein Species
Human
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Purity
>95% as determined by SDS-PAGE.
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Validations
Human GLB1/ELNR1 (His tag) recombinant protein
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