GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]

GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]

• Description: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Isoform 2 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. Defects in GLB1 are the cause of mucopolysaccharidosis type 4B (MPS4B) [MIM:253010]; also known as Morquio syndrome B. MPS4B is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life.
• Product Name Alternative: Acid beta galactosidase antibody Acid beta-galactosidase antibody Beta galactosidase 1 antibody Beta galactosidase antibody Beta-galactosidase antibody BGAL_HUMAN antibody EBP antibody EBP, included antibody Elastin receptor 1 (67kD) antibody Elastin receptor 1 67kDa antibody Elastin receptor 1 antibody Elastin receptor 1, included antibody Elastin-binding protein, included antibody ELNR1 antibody Galactosidase beta 1 antibody GLB 1 antibody GLB1 antibody Lactase antibody MPS4B antibody S-GAL, included antibody
• Abbreviation: Beta-galactosidase Acid beta-galactosidase (Lactase) Elastin receptor 1 GLB1 ELNR1
• CAS Number: 9000-83-3
• Swiss Prot: P16278 Human, P23780 Mouse,
• Cellular Locus: Lysosome and Cytoplasm.
• Host: Rabbit
• Species Reactivity: Human, Mouse, Rat
• Immunogen: Synthetic peptide within Human GLB1 aa 628-677 / 677.
• Isotype: IgG
• Clone: JE40-77
• Conjugation: Non-conjugated
• Type: Recombinant Rabbit monoclonal Antibody
• Applications: WB, FC, IHC-P
• Positive Control: SK-Br-3 cell lysate, Saos-2 cell lysate, U-87 MG cell lysate, MCF7 cell lysate, HepG2 cell lysate, K-562 cell lysate, Daudi cell lysate, NIH/3T3 cell lysate, Rat brain tissue lysate, mouse placenta tissue, SK-Br-3.
• Concentration: 1 mg/mL
• Dilution: WB: 1:1,000 ;FC: 1:500-1:1,000 ;IHC-P: 1:1,000
• Purity: Protein A affinity purified.
• Form: Liquid
• Buffer: 1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
• Molecular Weight: Predicted band size: 76 kDa
• Storage Conditions: Store at +4°C after thawing. Aliquot store at -20°C. Avoid repeated freeze/thaw cycles.
• Recombinant Antibody: Yes