| 產(chǎn)品編號(hào) | bsm-62323R |
| 英文名稱 | Beta galactosidase Recombinant Rabbit mAb |
| 中文名稱 | β半乳糖苷酶重組兔單抗 |
| 別 名 | EBP; ELNR1; Galactosidase beta 1; GLB1; Lactase; MPS4B. |
| 抗體來源 | Rabbit |
| 克隆類型 | Recombinant |
| 克 隆 號(hào) | 10A12 |
| 交叉反應(yīng) | Human,Mouse,Rat |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,Flow-Cyt=1:20-50
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 76 kDa |
| 檢測分子量 | 95 |
| 細(xì)胞定位 | 細(xì)胞漿 |
| 性 狀 | Liquid |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human beta Galactosidase |
| 亞 型 | IgG |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol. |
| 保存條件 | Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Function: 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. Subcellular Location: Isoform 1: Lysosome. Isoform 2: Cytoplasm, perinuclear region. Note=Localized to the perinuclear area of the cytoplasm but not to lysosomes. DISEASE: Defects in GLB1 are the cause of GM1-gangliosidosis type 1 (GM1G1) [MIM:230500]; also known as infantile GM1-gangliosidosis. GM1-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM1 gangliosides, glycoproteins and keratan sulfate primarily in neurons of the central nervous system. GM1G1 is characterized by onset within the irst three months of life, central nervous system degeneration, coarse facial features, hepatosplenomegaly, skeletal dysmorphology reminiscent of Hurler syndrome, and rapidly progressive psychomotor deterioration. Urinary oligosaccharide levels are high. It leads to death usually between the first and second year of life. Defects in GLB1 are the cause of GM1-gangliosidosis type 2 (GM1G2) [MIM:230600]; also known as late infantile/juvenile GM1-gangliosidosis. GM1G2 is characterized by onset between ages 1 and 5. The main symptom is locomotor ataxia, ultimately leading to a state of decerebration with epileptic seizures. 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 3 (GM1G3) [MIM:230650]; also known as adult or chronic GM1-gangliosidosis. GM1G3 is characterized by a variable phenotype. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Intellectual deficit can initially be mild or absent but progresses over time. Inheritance is autosomal recessive. 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. Similarity: Belongs to the glycosyl hydrolase 35 family. SWISS: P16278 Gene ID: 2720 Database links: GLB1前體實(shí)際分子量約85 kDa, 成熟蛋白約67 kDa. (JBC, Vol. 264, No. 34, Issue of Decemher 5, pp. 20655-20663,1989; JBC, Vol. 273, No. 11, Issue of March 13, pp. 6319–6326, 1998; J. Clin. Invest. 1993. 91:1198-1205; PLoS ONE 11(6): e0150210, 2016)(Jim 2020-09-07) |
| 產(chǎn)品圖片 |
25 ug total protein per lane of various lysates (see on figure) probed with Beta galactosidase monoclonal antibody, unconjugated (bsm-62323R) at 1:2000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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