Human beta-Galactosidase-1/GLB1 Alexa Fluor™ Plus 680‑conjugated Antibody

R&D Systems | Catalog # AF6464AFP680

R&D Systems

Key Product Details

Species Reactivity

Human

Applications

Western Blot

Label

Alexa Fluor Plus 680 (Excitation = 687 nm, Emission = 704 nm)

Antibody Source

Polyclonal Sheep IgG
View all beta-Galactosidase-1/GLB1 Primary Antibodies »

Product Specifications

Specificity

Detects human beta ‑Galactosidase‑1/GLB1 in direct ELISAs and Western blots.

Clonality

Polyclonal

Host

Sheep

Isotype

IgG

Applications

Application
Recommended Usage

Western Blot

Optimal dilution of this antibody should be experimentally determined.

Formulation, Preparation, and Storage

Formulation

Supplied 0.2 mg/mL in a saline solution containing BSA and Sodium Azide.

Shipping

The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

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).

References

  1. Hofer, D. et al. (2009) Hum. Mutat. 30:1214.
  2. Brunetti-Pierri N, and Scaglia F. (2008) Mol. Genet. Metab. 94:391.
  3. Santamaria, R. et al. (2007) J. Lipid Res. 48:2275.
  4. Prat, C. (2008) Joint Bone Spine, 75:495.
  5. Zhang, S. et al. (2000) Biochem. J. 348:621.
  6. Pshezhetsky, A.V. and Ashmarina, M. (2001) Prog. Nucleic Acid Res. Mol. Biol. 69:81.
  7. Salvatore P, et al. (1998) J. Biol. Chem. 273:6319.

Alternate Names

betaGalactosidase1, EBP, ELNR1, GLB1, Lactase

Entrez Gene IDs

2720 (Human)

Gene Symbol

GLB1

UniProt

AAA51819

Additional beta-Galactosidase-1/GLB1 Products

Product Documents

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

Product Specific Notices


This product is provided under an intellectual property license from Life Technologies Corporation. The transfer of this product is conditioned on the buyer using the purchased product solely in research conducted by the buyer, excluding contract research or any fee for service research, and the buyer must not (1) use this product or its components for (a) diagnostic, therapeutic or prophylactic purposes; (b) testing, analysis or screening services, or information in return for compensation on a per-test basis; or (c) manufacturing or quality assurance or quality control, and/or (2) sell or transfer this product or its components for resale, whether or not resold for use in research. For information on purchasing a license to this product for purposes other than as described above, contact Life Technologies Corporation, 5781 Van Allen Way, Carlsbad, CA 92008 USA or outlicensing@thermofisher.com.

For research use only

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