货号：AB1211-5MG

品牌：Merck Millipore

规格：

目录价：￥2492.00

市场价格：￥2118.20

会员价格：￥1993.60

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-β Galactosidase Antibody | AB1211-5MG View All» Replaces: AB1211 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Recognizes Beta-Galactosidase (E. coli). Monospecific by IEP. Some cross reactivity with Beta Galactosidase from other species may occur. View All» Immunogen: Beta-Galactosidase (E. coli). View All» Species Reactivity:

• Vertebrates

• E. coli
  View All» 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. View All» Control: Proteins tagged with beta galactosidase View All» Purification Method: Ammonium sulfate precipitation and DEAE-cellulose chromatography View All» 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. View All» 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. View All» UniProt Number: P16278 View All» Entrez Gene Number:
  • NM_000404.2

  • NM_001079811.1
    View All» Gene Symbol:
    • GLB1

    • Lactase

    • S-Gal

    • ELNR1

    • EBP
      View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
      • ELISA

      • Immunohistochemistry

      • Immunoprecipitation

      • Western Blotting
        View All» 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] View All» 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. View All» Brand Family: Chemicon View All» Product Name: Anti-β Galactosidase Antibody | AB1211-5MG View All» Concentration: 10 mg/mL View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 5 mg View All» Format: Purified View All» Host: Rabbit View All»
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