Human beta-Galactosidase-1/GLB1 Antibody Summary
Met1-Val677
Accession # P16278
Applications
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Scientific Data
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Detection of Human beta ‑Galactosidase‑1/GLB1 by Western Blot. Western blot shows lysates of JEG-3 human epithelial choriocarcinoma cell line and KG-1 human acute myelogenous leukemia cell line. PVDF membrane was probed with 1 µg/mL of Mouse Anti-Human beta -Galactosidase-1/GLB1 Monoclonal Antibody (Catalog # MAB6464) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # HAF007). A specific band was detected for beta -Galactosidase-1/GLB1 at approximately 64 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.
Reconstitution Calculator
Preparation and Storage
- 12 months from date of receipt, -20 to -70 °C as supplied.
- 1 month, 2 to 8 °C under sterile conditions after reconstitution.
- 6 months, -20 to -70 °C under sterile conditions after reconstitution.
Background: beta-Galactosidase-1/GLB1
GLB1, a 60-76 kDa (predicted) glycoprotein, 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.
Product Datasheets
Citations for Human beta-Galactosidase-1/GLB1 Antibody
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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Lysosome-targeted beta-galactosidase negatively regulates neuraminidase 1 (NEU1) and promotes NEU1 deficiency in GM1 gangliosidosis
Authors: AR Luu, H Wong, V Agrawal, N Wise, B Handyside, MJ Lo, G Pacheco, JB Felix, A Giaramita, A d'Azzo, J Vincelette, S Bullens, S Bunting, TM Christians, C Hague, JH Lebowitz, G Yogalingam
J. Biol. Chem., 2020-07-28;0(0):.
Species: Human
Sample Types: Cell Lysates
Applications: Western Blot -
Bi-functional IgG-lysosomal enzyme fusion proteins for brain drug delivery
Authors: RJ Boado, JZ Lu, EK Hui, H Lin, WM Pardridge
Sci Rep, 2019-12-09;9(1):18632.
Species: Chinese Hamster
Sample Types: Cell Lystates
Applications: Western Blot
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