Special Offers
Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| Vrt, E. coli | ELISA, IHC, IP, WB | Rb | Purified | Polyclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | AB1211-100UL |
| Brand Family | Chemicon® |
| Trade Name |
|
| Description | Anti-β Galactosidase Antibody |
| Product Information | |
|---|---|
| Format | Purified |
| Control |
|
| Presentation | Immunoglobulin Purified by delipidation, salt fractionation and ion-exchange chromatography. Liquid in 0.02M Potassium Phosphate buffer, 0.15M Sodium Chloride, with 0.01% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | Anti-β Galactosidase Antibody is an antibody against β Galactosidase for use in ELISA, Immunohistochemistry, Immunoprecipitation, Western Blotting. |
| Key Applications |
|
| Application Notes | ELISA: 1:10,000-1:50,000 when assayed against 1 μg of Beta Galactosidase in a standard sandwich ELISA. Immunoblotting: 1:2000-1:10,000 on beta-galactosidase fusion proteins. Immunohistochemistry: 1:1,000-1:5000 on tissue fixed with 4% paraformaldehyde. Immunoprecipitation Optimal working dilutions must be determined by end user. |
| Biological Information | |
|---|---|
| Immunogen | Beta-Galactosidase (E. coli). |
| Concentration | Please refer to the Certificate of Analysis for the lot-specific concentration. |
| Host | Rabbit |
| Specificity | Recognizes Beta-Galactosidase (E. coli). Monospecific by IEP. Some cross reactivity with Beta Galactosidase from other species may occur. |
| Species Reactivity |
|
| Antibody Type | Polyclonal Antibody |
| Entrez Gene Number |
|
| Entrez Gene Summary | The GLB1 gene encodes beta-galactosidase-1 (EC 3.2.1.23), a lysosomal hydrolase that cleaves the terminal beta-galactose from ganglioside substrates and other glycoconjugates (Yoshida et al., 1991 [PubMed 1907800]). Beta-galactosidase also occurs in a complex with neuraminidase (NEU1; MIM 608272) and protective protein/cathepsin A (PPCA; MIM 256540), which is a component of certain cell surface receptors (Hinek, 1996 [PubMed 8922281]). See also galactosylceramidase (GALC; MIM 606890) (EC 3.2.1.46), a genetically distinct beta-galactosidase that is involved in the catabolism of other lipid compounds.[supplied by OMIM] |
| Gene Symbol |
|
| Purification Method | Ammonium sulfate precipitation and DEAE-cellulose chromatography |
| UniProt Number |
|
| UniProt Summary | FUNCTION: SwissProt: P16279 # This protein has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non- integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers.| P16278 # Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. SIZE: 546 amino acids; 60552 Da SUBCELLULAR LOCATION: Cytoplasm, perinuclear region. Note=Localized to the perinuclear area of the cytoplasm but not to lysosomes. DOMAIN: SwissProt: P16279 DISEASE: SwissProt: P16278 # Defects in GLB1 are the cause of GM1-gangliosidosis type I [MIM:230500]; also known as infantile GM1-gangliosidosis. This autosomal recessive disorder is characterized by the accumulation in visceral tissues, and ultimately excessive excretion in the urine, of beta-linked galactose-terminal oligosaccharides. Patients show central nervous system degeneration, and the coarse facial features, hepatosplenomegaly and skeletal dysmorphology reminiscent of Hurler syndrome. The infantile form is rapidly progressive leading to death usually between the first and second year. & Defects in GLB1 are the cause of GM1-gangliosidosis type II [MIM:230600]; also known as late infantile/juvenile type GM1- gangliosidosis. Patients do not display the skeletal changes associated with the infantile form, but they nonetheless excrete elevated amounts of beta-linked galactose-terminal oligosaccharides. Inheritance is autosomal recessive. & Defects in GLB1 are the cause of GM1-gangliosidosis type III [MIM:230650]; also known as adult or chronic GM1- gangliosidosis. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Inheritance is autosomal recessive. & Defects in GLB1 are the cause of mucopolysaccharidosis IV B (MPS4B) [MIM:253010]; also known as Morquio syndrome B. MPS4B is a rare autosomal recessive disorder characterized by severe bone deformities without CNS involvement. SIMILARITY: SwissProt: P16279 ## Belongs to the glycosyl hydrolase 35 family. | P16278 ## Belongs to the glycosyl hydrolase 35 family. |
| Product Usage Statements | |
|---|---|
| Usage Statement |
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| Storage and Shipping Information | |
|---|---|
| Storage Conditions | Maintain for 2 years at -20°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. |
| Packaging Information | |
|---|---|
| Material Size | 100 µL |