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Recombinant Human β-Galactosidase/GLB1 Protein (His Tag)
SKU: PKSH033267-50
Recombinant Human β-Galactosidase/GLB1 Protein (His Tag)
| SKU # | PKSH033267 |
| Expression Host | HEK293 Cells |
Description
| Synonyms | Acid Beta-Galactosidase, Beta-Galactosidase, ELNR1, Elastin Receptor 1, GLB1, Lactase |
| Species | Human |
| Expression Host | HEK293 Cells |
| Sequence | Leu24-Val677 |
| Accession | P16278 |
| Calculated Molecular Weight | 74.6 kDa |
| Observed Molecular Weight | 90 kDa |
| Tag | C-His |
| Bio-activity | Not validated for activity |
Properties
| Purity | > 95 % as determined by reducing SDS-PAGE. |
| Endotoxin | < 1.0 EU per μg of the protein as determined by the LAL method. |
| Storage | Store at < -20°C, stable for 6 months. Please minimize freeze-thaw cycles. |
| Shipping | This product is provided as liquid. It is shipped at frozen temperature with blue ice/gel packs. Upon receipt, store it immediately at < - 20°C. |
| Formulation | Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0. |
| Reconstitution | Not Applicable |
Background
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.