β-半乳糖苷酶1/β-骋补濒/弹性蛋白受体1抗体
规格：1尘驳/1尘濒
英文名： GLB1
别名： Acid beta galactosidase; Acid beta-galactosidase; Beta galactosidase 1; Beta galactosidase; Beta-galactosidase; BGAL_HUMAN; EBP; EBP, included; Elastin receptor 1 (67kD); Elastin receptor 1 67kDa; Ela
分子量： 73kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human GLB1
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆
β-半乳糖苷酶1/β-骋补濒/弹性蛋白受体1抗体产物介绍：background: The human b-galactosidase gene, known as the LacZ gene, maps to chromosome 3p21.33 and encodes a 677 amino acid protein with an optimum functional pH range of 6 to 8. Catalytically active b-galactosidaseis (b-Gal) is a tetramer of four identical subunits, each with an active site, which can independently catalyze the cleavage of terminal galactose. Monovalent cations have a stimulatory effect on the enzymatic reaction, which likely involves a galactosyl-enzyme complex intermediate. b-Gals are widespread in animals, microorganisms and plants. The LacZ gene is widely used as a reporter gene with a variety of colored or fluorescent compounds capable of being produced from appropriate substrates, such as Xgal, which produces a blue color. For this reason, LacZ is incorporated into numerous plasmid vectors as a marker. 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: Lysosome and Cytoplasm > perinuclear region. 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 first 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 β-半乳糖苷酶1/β-骋补濒/弹性蛋白受体1抗体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 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 storageβ-半乳糖苷酶1/β-骋补濒/弹性蛋白受体1抗体 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. Database links: UniProtKB/Swiss-Prot: P16278.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
β-半乳糖苷酶1/β-骋补濒/弹性蛋白受体1抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 转运蛋白 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid