145475
GLB1 (beta-Galactosidase-1, EBP, ELNR1, Lactase)
Size
£882.00
- SKU:
- 145475
- Purity:
- Purified by Protein A affinity chromatography from hybridoma culture supernatant.
- Storage Conditions:
- -20°C
- Supplier:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Recombinant protein corresponding to Met1-Val677 from human GLB1, expressed in CHO cells.
- Clone:
- 810226
- Format:
- Supplied as a lyophilized powder in PBS, 5% trehalose. Reconstitute with 200ul sterile PBS.
- Extra Details:
- GLB1, a 60-76kD (predicted) glycoprotein, is a lysosomal betagalactosidase that hydrolyzes the terminal betagalactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis 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 beta-galactosidase 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 the two related diseases (5). In lysosome, the mature beta-galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex (6). An alternative splicing at the RNA level of GLB1 results a catalytically inactive beta-galactosidase (also called elastin-binding protein) that plays an important role in vascular development (7). Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: Western Blot: 1ug/ml with JEG‑3 human epithelial choriocarcinoma cell line and KG‑1 human acute myelogenous leukemia cell line Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.
- Shipping Conditions:
- Blue Ice