氨基己糖苷酶Aα(HEXa)活性蛋白

Active Hexosaminidase A Alpha (HEXa)

TSD; Hex-A; Tay Sachs Disease; GM2 Gangliosidosis; Beta-Hexosaminidase Subunit Alpha; Beta-N-Acetylhexosaminidase Subunit Alpha; N-Acetyl-Beta-Glucosaminidase Alpha

  • 氨基己糖苷酶Aα(HEXa)活性蛋白 产品包装(模拟)
  • 氨基己糖苷酶Aα(HEXa)活性蛋白 产品包装(模拟)
  • APA195Mu01.jpg SDS-PAGE图
  • 氨基己糖苷酶Aα(HEXa)活性蛋白 Figure. Western Blot; Sample: Recombinant HEXa, Mouse.
  • Certificate 通过ISO 9001、ISO 13485质量体系认证

活性实验

Hexosaminidase A Alpha (HEXa) is a lysosomal enzyme. There are three predominant isoenzymes: hexosaminidase A, B and S. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. The enzymes are composed of two alpha and/or beta subunits, which are coded by HEXA and HEXB genes, respectively. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit which contains a key residue, Arg-424 is able to hydrolyze GM2 gangliosides. Hexosaminidase A (alpha polypeptide) plays a critical role in the brain and spinal cord (central nervous system). Besides, Hexosaminidase B Beta (HEXB) has been identified as an interactor of HEXA, thus a binding ELISA assay was conducted to detect the interaction of recombinant mouse HEXA and recombinant mouse HEXB. Briefly, HEXA were diluted serially in PBS, with 0.01% BSA (pH 7.4). Duplicate samples of 100uL were then transferred to HEXB-coated microtiter wells and incubated for 2h at 37℃. Wells were washed with PBST and incubated for 1h with anti-HEXA pAb, then aspirated and washed 3 times. After incubation with HRP labelled secondary antibody, wells were aspirated and washed 3 times. With the addition of substrate solution, wells were incubated 15-25 minutes at 37℃. Finally, add 50µL stop solution to the wells and read at 450nm immediately. The binding activity of of HEXA and HEXB was shown in Figure 1, and this effect was in a dose dependent manner.

用法

Reconstitute in 20mM Tris, 150mM NaCl (pH8.0) to a concentration of 0.1-1.0 mg/mL. Do not vortex.

储存

避免反复冻融。2-8°C不超过一个月,-80°C不超过12个月。

稳定性

热稳定性以损失率显示。损失率是由加速降解试验决定,具体方法如下:在37°C孵育48小时,没有显著的降解或者沉淀产生。保质期内,在适当的条件下存储,损失率低于5%。

相关产品

编号 适用物种:Mus musculus (Mouse,小鼠) 应用(仅供研究使用,不用于临床诊断!)
APA195Mu01 氨基己糖苷酶Aα(HEXa)活性蛋白 Cell culture; Activity Assays.
RPA195Mu01 氨基己糖苷酶Aα(HEXa)重组蛋白 Positive Control; Immunogen; SDS-PAGE; WB.
RPA195Mu02 氨基己糖苷酶Aα(HEXa)重组蛋白 Positive Control; Immunogen; SDS-PAGE; WB.
PAA195Mu01 氨基己糖苷酶Aα(HEXa)多克隆抗体 WB; IHC; ICC; IP.
PAA195Mu02 氨基己糖苷酶Aα(HEXa)多克隆抗体 WB; IHC; ICC; IP.
SEA195Mu 氨基己糖苷酶Aα(HEXa)检测试剂盒(酶联免疫吸附试验法) Enzyme-linked immunosorbent assay for Antigen Detection.
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