Human beta -Galactosidase-1/GLB1 Antibody

  
  • Species Reactivity
    Human
  • Specificity
    Detects human beta ‑Galactosidase‑1/GLB1 in direct ELISAs and Western blots.
  • Source
    Polyclonal Sheep IgG
  • Purification
    Antigen Affinity-purified
  • Immunogen
    Chinese hamster ovary cell line CHO-derived recombinant human beta ‑Galactosidase‑1/GLB1
    Leu24-Val677
    Accession # AAA51819
  • Formulation
    Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.
  • Label
    Unconjugated
Applications
  •  
    Recommended
    Concentration
    Sample
  • Western Blot
    1 µg/mL
    See below
  • Simple Western
    10 µg/mL
    See below
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.
Data Examples

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
  • Reconstitution
    Sterile PBS to a final concentration of 0.2 mg/mL.
  • Shipping
    The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C
  • Stability & Storage
    Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
    • 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).
  • 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.
  • Entrez Gene IDs:
    2720 (Human)
  • Alternate Names:
    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
Related Research Areas
Isotype Controls
Description Application Cat# Citations Images  

Normal Sheep IgG Control

Ctrl 5-001-A 9
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....
Secondary Antibodies
Description Application Cat# Citations Images  

Sheep IgG HRP-conjugated Antibody

WB, Simple Western HAF016 14  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL557-conjugated Antibody

Flow, IHC, ICC NL010 3
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL637-conjugated Antibody

Flow, IHC, ICC NL011 5
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG NorthernLights™ NL493-conjugated Antibody

Flow, IHC, ICC NL012 2
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG (H+L) PE-conjugated Antibody

Flow F0126  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG Biotinylated Antibody

WB BAF016 2
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG (H+L) APC-conjugated Antibody

Flow F0127  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Sheep IgG VisUCyte HRP Polymer Antibody

IHC VC006  
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG (H+L) Fluorescein-conjugated Antibody

Flow F0125 1
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Donkey Anti-Sheep IgG (H+L) PerCP-conjugated Antibody

Flow F0128
View Sizes & Prices
Catalog# Size Price Stock Quantity
Loading Information....

Have you used Human beta -Galactosidase-1/GLB1 Antibody?

Submit a review and receive a $25US/€18/£15/$25CAN amazon gift card if you include an image - $10US/€7/£6/$10CAN Amazon card for reviews without an image. Limited to verified customers in USA, Canada and Europe.

Order Details

Contact Information

Calculators

Reconstitution Calculator

The reconstitution calculator allows you to quickly calculate the volume of a reagent to reconstitute your vial. Simply enter the mass of reagent and the target concentration and the calculator will determine the rest.

=
÷
R&D Systems Guarantee

Monoclonal
Antibody
Panels For
Screening

Learn More
Monoclonal Antibodies

Feedback

Customer Information