Human beta -Galactosidase-1/GLB1 Antibody

  
  • Species Reactivity
    Human
  • Specificity
    Detects human beta ‑Galactosidase‑1/GLB1 in direct ELISAs.
  • Source
    Monoclonal Mouse IgG2A Clone # 810226
  • Purification
    Protein A or G purified from hybridoma culture supernatant
  • Immunogen
    Chinese hamster ovary cell line CHO-derived recombinant human beta ‑Galactosidase‑1/GLB1
    Met1-Val677
    Accession # P16278
  • 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
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 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.
Preparation and Storage
  • Reconstitution
    Sterile PBS to a final concentration of 0.5 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
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Mouse IgG2A Isotype Control

Ctrl MAB003 32  
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Mouse IgG2A Isotype Control

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Mouse IgG2A Isotype Control

Ctrl MAB003R
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Secondary Antibodies
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Mouse IgG HRP-conjugated Antibody

WB HAF007 26  
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Mouse F(ab)2 IgG (H+L) PE-conjugated Antibody

Flow F0102B 10  
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Donkey Anti-Mouse IgG NorthernLights™ NL557-conjugate Antibody

Flow, IHC, ICC NL007 9
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Donkey Anti-Mouse IgG Biotinylated Antibody

WB BAF018 1
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Mouse IgG HRP-conjugated Antibody

WB HAF018 9  
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Donkey Anti-Mouse IgG NorthernLights™ NL637-conjugated Antibody

Flow, IHC, ICC NL008 8
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Mouse F(ab)2 IgG (H+L) APC-conjugated Antibody

Flow F0101B 2  
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Donkey Anti-Mouse IgG NorthernLights™ NL493-conjugated Antibody

Flow, IHC, ICC NL009 5
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Mouse F(ab)2 IgG (H+L) Fluorescein-conjugated Antibody

Flow F0103B 5  
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Mouse IgG Antibody

WB AF007 4
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Goat Anti-Mouse IgG Biotinylated Antibody

WB BAF007 4
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Mouse/Rabbit IgG VisUCyte HRP Polymer Antibody

IHC VC002  
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Rat Anti-Mouse IgG2A PE-conjugated Antibody

Flow F0129 1  
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Mouse IgG VisUCyte HRP Polymer Antibody

IHC VC001  
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Goat Anti-Mouse IgG Fc Affinity Purified Polyclonal Ab

G-202-C 2
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Mouse F(ab)2 IgG (H+L) PerCP-conjugated Antibody

Flow F0114  
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Rat Anti-Mouse IgG2A APC-conjugated Antibody

Flow F0130  
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Mouse IgG2A Antibody

Flow MAB0032  
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Rat Anti-Mouse IgG2A PerCP-conjugated Antibody

Flow F0131  
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