Key Product Details

Species Reactivity

Validated:

Human

Cited:

Human, Hamster - Cricetulus (Chinese Hamster)

Applications

Validated:

Western Blot

Cited:

Western Blot

Label

Unconjugated

Antibody Source

Monoclonal Mouse IgG2A Clone # 810226
View all beta-Galactosidase-1/GLB1 Primary Antibodies »

Product Specifications

Immunogen

Chinese hamster ovary cell line CHO-derived recombinant human beta ‑Galactosidase‑1/GLB1
Met1-Val677
Accession # P16278

Specificity

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

Clonality

Monoclonal

Host

Mouse

Isotype

IgG2A

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 Mouse Anti-Human beta -Galactosidase-1/GLB1 Monoclonal Antibody (Catalog # MAB6464) followed by HRP-conjugated Anti-Mouse IgG Secondary Antibody (Catalog # HAF007). A specific band was detected for beta -Galactosidase-1/GLB1 at approximately 64 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Applications for Human beta-Galactosidase-1/GLB1 Antibody

Application
Recommended Usage

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

Protein A or G purified from hybridoma culture supernatant

Reconstitution

Sterile PBS to a final concentration of 0.5 mg/mL. For liquid material, refer to CoA for concentration.

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.

Calculators

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.

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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

P16278 (Human)

Additional beta-Galactosidase-1/GLB1 Products

Product Documents for Human beta-Galactosidase-1/GLB1 Antibody

Certificate of Analysis

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Note: Certificate of Analysis not available for kit components.

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

For research use only

Citations for Human beta-Galactosidase-1/GLB1 Antibody

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