beta-Galactosidase-1/GLB1 Antibody [Alexa Fluor® 647]

• Reactivity: Hu
• Applications: WB
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor 647

beta-Galactosidase-1/GLB1 Antibody [Alexa Fluor® 647] Summary

• Immunogen: Chinese hamster ovary cell line CHO-derived recombinant human β‑Galactosidase‑1/GLB1
  Leu24-Val677
  Accession # AAA51819
• Specificity: Detects human beta ‑Galactosidase‑1/GLB1 in direct ELISAs and Western blots.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Purity Statement: Antigen Affinity-purified

Applications/Dilutions

• Dilutions:
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for beta-Galactosidase-1/GLB1 Antibody [Alexa Fluor® 647]

• Acid beta-galactosidase
• beta-galactosidase
• betaGalactosidase1
• beta-Galactosidase-1
• EBP
• EC 3.2.1.23
• elastin receptor 1 (67kD)
• Elastin receptor 1
• elastin receptor 1, 67kDa
• ELNR1
• galactosidase, beta 1
• GLB1
• Lactase
• MPS4B

Background

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).

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.