Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF
Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF Summary
Product Specifications
Analysis
Product Datasheets
Carrier Free
CF stands for Carrier Free (CF). We typically add Bovine Serum Albumin (BSA) as a carrier protein to our recombinant proteins. Adding a carrier protein enhances protein stability, increases shelf-life, and allows the recombinant protein to be stored at a more dilute concentration. The carrier free version does not contain BSA.
In general, we advise purchasing the recombinant protein with BSA for use in cell or tissue culture, or as an ELISA standard. In contrast, the carrier free protein is recommended for applications, in which the presence of BSA could interfere.
6464-GH
| Formulation | Supplied as a 0.2 μm filtered solution in Tris and NaCl. |
| Shipping | The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below. |
| Stability & Storage: | Store the unopened product at -70 °C. Use a manual defrost freezer and avoid repeated freeze-thaw cycles. Do not use past expiration date. |
Scientific Data
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Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF (Catalog # 6464-GH) removes terminal galactose residues from various glycolipids, glycoproteins, and glycosaminoglycans.
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Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF (Catalog # 6464-GH) is measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl-beta -D-galactopyranoside.
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2 μg/lane of Recombinant Human beta ‑Galactosidase‑1/GLB1 Protein (Catalog # 6464-GH) was resolved with SDS-PAGE under reducing (R) and non-reducing (NR) conditions and visualized by Coomassie® Blue staining, showing bands at 70-95 kDa.
Reconstitution Calculator
Background: beta-Galactosidase-1/GLB1
GLB1 is a lysosomal beta -galactosidase that hydrolyzes the terminal beta -galactose 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).
- Hofer, D. et al. (2009) Hum. Mutat. 30:1214.
- Brunetti-Pierri N, and Scaglia F. (2008) Mol. Genet. Metab. 94:391.
- Santamaria, R. et al. (2007) J. Lipid Res. 48:2275.
- Prat, C. (2008) Joint Bone Spine, 75:495.
- Zhang, S. et al. (2000) Biochem. J. 348:621.
- Pshezhetsky, A.V. and Ashmarina, M. (2001) Prog. Nucleic Acid Res. Mol. Biol. 69:81.
- Salvatore P, et al. (1998) J. Biol. Chem. 273:6319.
Citations for Recombinant Human beta-Galactosidase-1/GLB1 Protein, CF
R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions.
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Citations: Showing 1 - 2
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Dysregulated non-coding telomerase RNA component and associated exonuclease XRN1 in leucocytes from women developing preeclampsia-possible link to enhanced senescence
Authors: T Lekva, MCP Roland, ME Estensen, ER Norwitz, T Tilburgs, T Henriksen, J Bollerslev, KR Normann, P Magnus, OK Olstad, P Aukrust, T Ueland
Scientific Reports, 2021-10-05;11(1):19735.
Species: Human
Sample Types: Plasma
Applications: ELISA Standard -
Fluorimetric assay with a novel substrate for quantification of galactocerebrosidase activity in dried blood spot specimens
Authors: AJ Ullal, H Pham, R Singh, P Ross, CA Graham, SM Norton, MH Nuffer, DS Burns, AE Eckhardt, M Escolar, D Bali, VK Pamula
Pract Lab Med, 2019-10-16;18(0):e00141.
Species: Human
Sample Types: Protein
Applications: Bioassay
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