β半乳糖苷酶抗体

β半乳糖苷酶抗体英文名称  Anti-Beta galactosidase 

中文名称  β半乳糖苷酶抗体 

别    名  beta-Galactosidase; Beta D galactosidase; Beta gal; Beta galactosidase; EC 3.2.1.23; ECK0341; JW0335; lactase; lacZ; BGAL_HUMAN; Acid beta-galactosidase; Lactase; Elastin receptor 1. 

浓    度  1mg/1ml 

规 格  0.2ml/200μg     

纯化的抗体可通过不同的途径获取，有些β半乳糖苷酶抗体可通过下述方法制备或从商家购买。从商家购买的抗体，通常附有正确的储存方法。

1）工作液应在4℃下融化并存放，可能稳定达数月。

2）如果没有特殊原因而避免使用叠氮钠，亦可加入叠氮钠，浓度为0．02％。将纯化的抗体样本分装成合适的体积，于-20℃保存。

3）纯化的抗体溶液应以较高的浓度(如lmg／m1)在中性pH下保存。：常用的抗体储存浓度高达l0mg/ml。较低浓度的抗体冻存前应浓缩。所有标准的浓缩方法(如超滤法)，皆可使用。还有一个简单的方法是用蛋白A或蛋白G亲和柱来浓缩溶液。如果纯化的抗体不是用于标记，可将它们以较低浓度储存于加有1％BSA的溶液中。

4）经纯化制备的抗体在常用的缓冲液中是稳定的。其DH应保持在中性左右。如果pH在7-8之间，即使保存多年，对抗体也无损害。多数情况下，盐浓度适于保持在0-150mmol/L之间，但在长期存放的抗体中，盐溶液浓度高达500mmol/L时，对β半乳糖苷酶抗体能有损害。如果没有其他说明．律议用PBS或50mmol/LTris(DH8．0)溶液长期存放抗体。  

抗体来源  Rabbit  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse

产品类型  一抗    

研究领域  细胞生物 免疫学  

蛋白分子量  predicted molecular weight: 71kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human Beta galactosidase (221-290aa) 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4 

产品应用   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.  

保存条件  Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. 

Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

β半乳糖苷酶抗体产品介绍 Beta galactosidase is coded by a gene (lac z) in the lac operon of Escherichia coli. It is a metalloenzyme that splits lactose into glucose and galactose. It hydrolyzes terminal, non-reducing beta-D-galactose residues in beta-D-galactosides. Activation by cations seems to be substrate dependent. K+, Na+, NH4+, Rb+, Cs+ and Mn++ all activate enzyme activity based upon the substrate used.

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.

Database links : UniProtKB/Swiss-Prot: P16278.2

β-Galactosidase.