Human beta-Galactosidase-1/GLB1 Antibody Summary
Accession # AAA51819
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.
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 Sheep Anti-Human beta -Galactosidase-1/GLB1 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6464) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). Specific bands were detected for beta -Galactosidase-1/GLB1 at approximately 60 kDa and 80 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 8.
Detection of Human beta ‑Galactosidase‑1/GLB1 by Simple WesternTM. Simple Western lane view shows lysates of JEG-3 human epithelial choriocarcinoma cell line, loaded at 0.2 mg/mL. Specific bands were detected for beta -Galactosidase-1/GLB1 at approximately 71 & 110 kDa (as indicated) using 10 µg/mL of Sheep Anti-Human beta -Galactosidase-1/GLB1 Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6464) followed by 1:50 dilution of HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). This experiment was conducted under reducing conditions and using the 12-230 kDa separation system.
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.
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.
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