Human beta-Galactosidase-1/GLB1 Antibody

R&D Systems, part of Bio-Techne | Catalog # AF6464

R&D Systems, part of Bio-Techne
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AF6464
AF6464-SP
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Key Product Details

Species Reactivity

Human

Applications

Simple Western, Western Blot

Label

Unconjugated

Antibody Source

Polyclonal Sheep IgG

View all beta-Galactosidase-1/GLB1 Antibodies »

Product Specifications

Immunogen

Chinese hamster ovary cell line CHO-derived recombinant human beta‑Galactosidase‑1/GLB1
Leu24-Val677
Accession # AAA51819

Specificity

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

Clonality

Polyclonal

Host

Sheep

Isotype

IgG

Scientific Data Images for Human beta-Galactosidase-1/GLB1 Antibody

Detection of Human beta-Galactosidase-1/GLB1 antibody by Western Blot.

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 antibody by Simple WesternTM.

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.

Applications for Human beta-Galactosidase-1/GLB1 Antibody

Application
Recommended Usage

Simple Western

10 µg/mL
Sample: JEG‑3 human epithelial choriocarcinoma cell line

Western Blot

1 µg/mL
Sample: JEG‑3 human epithelial choriocarcinoma cell line and KG‑1 human acute myelogenous leukemia cell line

Formulation, Preparation, and Storage

Purification

Antigen Affinity-purified

Reconstitution

Sterile PBS to a final concentration of 0.2 mg/mL. For liquid material, refer to CoA for concentration.

Reconstitution Buffer Available:
Size / Price
Qty

Formulation

Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

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.

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 for Human beta-Galactosidase-1/GLB1 Antibody

Product Datasheets

Certificate of Analysis

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Product Specific Notices for Human beta-Galactosidase-1/GLB1 Antibody

For research use only

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