Human beta-Galactosidase-1/GLB1 Antibody
R&D Systems, part of Bio-Techne | Catalog # MAB6464
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
Product Specifications
Immunogen
Chinese hamster ovary cell line CHO-derived recombinant human beta‑Galactosidase‑1/GLB1
Met1-Val677
Accession # P16278
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 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
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.
Background: beta-Galactosidase-1/GLB1
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
- Hofer, D. et al. (2009) Hum. Mutat. 30:1214.
- Brunetti-Pierri N, and Scaglia F. (2008) Mol. Genet. Metab. 94:391.
- Santamaria, R. et al. (2007) J. Lipid Res. 48:2275.
- Prat, C. (2008) Joint Bone Spine, 75:495.
- Zhang, S. et al. (2000) Biochem. J. 348:621.
- Pshezhetsky, A.V. and Ashmarina, M. (2001) Prog. Nucleic Acid Res. Mol. Biol. 69:81.
- 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
Additional beta-Galactosidase-1/GLB1 Products
Product Documents for Human beta-Galactosidase-1/GLB1 Antibody
Product Specific Notices for Human beta-Galactosidase-1/GLB1 Antibody
For research use only
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