GBA (基因名), Lysosomal acid glucosylceramidase (蛋白名), glcm_human.
产品名称:
Human GBA/ Lysosomal acid glucosylceramidase Recombinant Protein
葡糖神经酰胺
货号:
R1448h
商标:
EIAab®
监管等级:
别名:
Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Cholesterol glucosyltransferase, Cholesteryl-beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Imiglucerase, Beta-GC, SGTase, Lysosomal acid GCase, GC, GLUC
序列号:
P04062
来源:
E.coli
种属:
Human
标签:
His
序列:
81-400aa
预估分子量:
35.2 kDa (monomer)
纯度:
Greater than 95% by SDS-PAGE
浓度:
Reconstitution Dependent
形态:
Liquid
内毒素水平:
Please contact protein@eiaab.com The technician for more information.
应用:
存储缓冲液:
50mM NaH2PO4, 500mM NaCl Buffer with 500mM Imidazole, 10%glycerol(PH8.0)
存储:
Store at -20°C. (Avoid repeated freezing and thawing.)
研究领域:
Cardiovascular
R&D 技术数据
The PCR product of human GBA gene was determined by 1% Agarose stained with EB.
Recombinant human GBA protein was determined by 12% SDS-PAGE stained with Coomassie Blue under reducing conditions.
基因位点
GBA Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
通用注释
亚单元:
Interacts with saposin-C (PubMed:10781797). Interacts with SCARB2 (PubMed:18022370). Interacts with TCP1 (PubMed:21098288). May interact with SNCA; may inhibit the glucosylceramidase activity (PubMed:23266198). Interacts with GRN; this interaction prevents aggregation of GBA-SCARB2 complex via interaction with HSPA1A upon stress (PubMed:27789271).
功能:
Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed:24211208). Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates to the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Also plays a role in cholesterol metabolism (PubMed:24211208, PubMed:26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed:24211208, PubMed:26724485). The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed:26724485). Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol (PubMed:24211208, PubMed:26724485).
亚细胞位置:
Lysosome membrane
Peripheral membrane protein
Lumenal side
Interaction with saposin-C promotes membrane association (PubMed:10781797). Targeting to lysosomes occurs through an alternative MPR-independent mechanism via SCARB2 (PubMed:18022370).
该产品尚未在任何出版物中被引用。
[1].
"Multicenter analysis of glucocerebrosidase mutations in Parkinson's disease."
[2].
"Glucocerebrosidase mutations in clinical and pathologically proven Parkinson's disease."
[3].
"Mutations in the Parkinson's disease genes, Leucine Rich Repeat Kinase 2 (LRRK2) and Glucocerebrosidase (GBA), are not associated with essential tremor."
[5].
"Complete screening for glucocerebrosidase mutations in Parkinson disease patients from Greece."
[6].
"Mutations in GBA are associated with familial Parkinson disease susceptibility and age at onset."
[7].
"Association between Parkinson's disease and glucocerebrosidase mutations in Brazil."
[8].
"Molecular analysis of Turkish Gaucher disease patients: identification of novel mutations in glucocerebrosidase (GBA) gene."
[9].
"Genotype-phenotype correlations between GBA mutations and Parkinson disease risk and onset."
[1].
人葡糖神经酰胺(GBA)重组蛋白是否是无菌的?
蛋白试剂瓶和蛋白保存液是经过高压灭菌的,但也不能保证蛋白是完全无菌的。如果要求蛋白是无菌的,可以用0.2微米的滤器对蛋白进行过滤。
[2].
人葡糖神经酰胺(GBA)重组蛋白的保存缓冲液是什么?
纯化后的蛋白保存在PBS(58mM Na2HPO4, 17mM NaH2PO4, 68mM NaCl, pH7.4)里,并往里面加入500mM咪唑和10%甘油。
[3].
怎样确定人葡糖神经酰胺(GBA)重组蛋白的浓度?
蛋白浓度的确定没有一个统一的标准,这主要取决于蛋白的氨基酸序列。伊艾博是根据不同测试的组合来测定蛋白浓度。考马斯亮蓝法、BCA法、氨基酸序列和氨基酸全序列分析法等都用来测定蛋白浓度。
[4].
人葡糖神经酰胺(GBA)重组蛋白蛋白保存条件是怎样的?
蛋白应保存在 -20℃或 -80℃条件下,为了避免反复冻融,可以将蛋白分装成小份保存。
[5].
人葡糖神经酰胺(GBA)重组蛋白是否可以用于活体实验?
重组蛋白没有用于任何的活体实验,因此蛋白的活性和半衰期是不确定的。
[6].
人葡糖神经酰胺(GBA)重组蛋白的保质期是多久?
在适当的保存条件下,从购买之日起蛋白可以稳定保存6-12个月。适当的保存条件是:蛋白保存在-20°C o或 -80℃,保证蛋白的保存浓度高于0.1mg/ml,限制蛋白反复冻融的次数。我们公司常规的质量检测保证所有产品在销售时都有可接受的生物活性。但是我们不能控制终端用户蛋白的保存条件。如果产品在有效期内出现问题,请联系我们的技术支持。
[7].
你们蛋白和抗体的报价是怎么样的?
我们将根据你需要的蛋白和抗体的大小进行报价。
[8].
人葡糖神经酰胺(GBA)重组蛋白是否能够提供蛋白片段?
我们现有的人的蛋白的序列可以有很多。你可以选择你感兴趣的靶向部分,我们将会按您的需求提供蛋白和抗体。
[9].
人葡糖神经酰胺(GBA)重组蛋白的货期或发货时间一般是多长?
具体指标的货期需要确定。最快一周,最长可能一个月。
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