Human beta-Galactosidase-1/GLB1 Alexa Fluor® 700-conjugated Antibody
R&D Systems, part of Bio-Techne | Catalog # FAB6464N
Conjugate
Catalog #
Key Product Details
Species Reactivity
Human
Applications
Western Blot
Label
Alexa Fluor 700 (Excitation = 675-700 nm, Emission = 723 nm)
Antibody Source
Monoclonal Mouse IgG2A Clone # 810226
Product Specifications
Immunogen
Chinese hamster ovary cell line CHO-derived recombinant human β‑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
Applications
Application
Recommended Usage
Western Blot
Optimal dilution of this antibody should be experimentally determined.
Formulation, Preparation, and Storage
Purification
Protein A or G purified
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
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).
Alternate Names
betaGalactosidase1, EBP, ELNR1, GLB1, Lactase
Entrez Gene IDs
2720 (Human)
Gene Symbol
GLB1
UniProt
Additional beta-Galactosidase-1/GLB1 Products
Product Specific Notices
This product is provided under an agreement between Life Technologies Corporation and R&D Systems, Inc, and the manufacture, use, sale or import of this product is subject to one or more US patents and corresponding non-US equivalents, owned by Life Technologies Corporation and its affiliates. The purchase of this product conveys to the buyer the non-transferable right to use the purchased amount of the product and components of the product only in research conducted by the buyer (whether the buyer is an academic or for-profit entity). The sale of this product is expressly conditioned on the buyer not using the product or its components (1) in manufacturing; (2) to provide a service, information, or data to an unaffiliated third party for payment; (3) for therapeutic, diagnostic or prophylactic purposes; (4) to resell, sell, or otherwise transfer this product or its components to any third party, or for any other commercial purpose. Life Technologies Corporation will not assert a claim against the buyer of the infringement of the above patents based on the manufacture, use or sale of a commercial product developed in research by the buyer in which this product or its components was employed, provided that neither this product nor any of its components was used in the manufacture of such product. For information on purchasing a license to this product for purposes other than research, contact Life Technologies Corporation, Cell Analysis Business Unit, Business Development, 29851 Willow Creek Road, Eugene, OR 97402, Tel: (541) 465-8300. Fax: (541) 335-0354.
For research use only
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