活性重组蛋白 Recombinant Human GLB1 / ELNR1 / EBP / Lactase / MPS4B Protein　重组蛋白

研究方向

β-半乳糖苷酶 (GLB1) 蛋白作为一种酶，可从各种底物（包括神经节苷脂、糖蛋白和糖胺聚糖）中裂解 β 连接的末端半乳糖基残基。尽管缺乏直接的 β-半乳糖苷酶催化活性，GLB1 在弹性生成中发挥着重要的功能作用，有助于细胞外弹性纤维的形成和结缔组织的发育。有趣的是，GLB1 被确定为弹性蛋白结合蛋白 (EBP)，是成纤维细胞、平滑肌细胞、软骨细胞、白细胞和某些癌细胞类型上发现的非整联蛋白细胞表面受体的重要组成部分。在产生弹性蛋白的细胞中，GLB1 在细胞内与弹性蛋白原结合，作为回收分子伴侣，促进弹性蛋白原的分泌并支持其组装成弹性纤维。这种多方面的功能使 GLB1 在与组织发育和维护相关的酶促过程和结构作用中发挥关键作用。

种属

Human

表达宿主

Chinese Hamster Ovary cell CHO

Observed MW

70-95 kDa

标签

C-His

纯度

> 95% as determined by Tris-Bis PAGE.> 95% as determined by HPLC.

内毒素

< 0.1EU per μg by the LAL method.

生物活性

Measured by its ability to cleave a fluorogenic substrate, 4- Methylumbelliferyl-beta -D-galactopyranoside.The specific activity is >1,800 pmol/min/μg, as measured under the described conditions.

制剂

Lyophilized from 0.22um filtered solution in 20mM PB, 300mM NaCl (pH 6.5). Normally 5 % trehalose is added as protectant before lyophilization.

区间

1-677 aa

储存

Store at -20℃.Store the lyophilized protein at -20℃ to -80 ℃ up to 1 year from the date of receipt.
After reconstitution, the protein solution is stable at -20℃ for 3 months, at 2-8℃ for up to 1 week.未开盖的干粉蛋白在 -20°C至-80°C可保存12个月；
复溶之后，蛋白溶液在-20°C及以下可保存3个月，在2-8℃可保存1周。

复溶

Centrifuge the tube before opening. Reconstitute to a concentration of 0.1-0.5 mg/mL in sterile distilled water. Avoid vortex or vigorously pipetting the protein. For long term storage, it is recommended to add a carrier protein or stablizer (e.g. 0.1% BSA, 5% HSA, 10% FBS or 5% Trehalose), and aliquot the reconstituted protein solution to minimize free-thaw cycles.收到重组蛋白产品之后请检查蛋白冻干粉末是否贴于瓶底，如果粉末浮起，开盖之前请先低温离心。将蛋白用说明书中指定的缓冲液复溶至0.1-0.5 mg/mL（请注意蛋白复溶浓度不能低于0.1 mg/mL），室温平衡5-10 min保证充分溶解，复溶过程中请不要剧烈涡旋及吹打蛋白溶液。如需长期储存，建议复溶时添加载体蛋白或者稳定剂（如0.1% BSA, 5% HSA, 10% FBS 或者 5% 海藻糖），同时将复溶后的蛋白溶液按照需求进行分装，储存于-20°C至-80°C，随取随用，避免反复冻融。

背景信息

GLB1 is a lysosomal beta -galactosidase that hydrolyzes the terminal beta -galactose from ganglioside and keratan sulfate. Defects in this gene are the causes of lysosomal storage diseases for GM1-gangliosidosis and Morquio B syndrome (also known as mucopolysaccharidosis IVB) . In GM1 gangliosidosis, GM1 ganglioside accumulates in the neurons of the central nervous system, because of the deficiency (0±3% of normal) of lysosomal beta -galactosidase activity. GM1 gangliosidosis demonstrates varying degrees of clinical severity but is invariably fatal, and children with the most common and severe form of GM1 gangliosidosis usually die within 3 years of birth. Morquio B syndrome patients are neurologically normal, but display severe skeletal dysostosis multiplex because of an accumulation of keratan sulfate . More than 100 mutations have been identified for GLB1, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases. In lysosome, the mature beta -galactosidase protein associates with cathepsin A and neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of GLB1 results a catalytically inactive beta -galactosidase (also called elastin-binding protein) that plays an important role in vascular development .

Swiss Prot

P16278

别名

GFR alpha-like; GFRAL; GRAL; C6orf144