FITC标记的GTP结合蛋白SAR1B抗体
产品名称： FITC标记的GTP结合蛋白SAR1B抗体
英文名称： Anti-SAR1B/FITC
产品编号： HZ-19392R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-SAR1B/FITC Conjugated antibody
FITC标记的GTP结合蛋白SAR1B抗体
| 英文名称 | Anti-SAR1B/FITC |
| 中文名称 | FITC标记的GTP结合蛋白SAR1B抗体 |
| 别 名 | ANDD; CMRD; GTBPB; GTP binding protein SAR1b; GTP binding protein Sara; GTP-binding protein B; GTP-binding protein SAR1B; SAR1 gene homolog B (S. cerevisiae); SAR1 homolog B; SAR1a gene homolog 2; SAR1B; SAR1B_HUMAN; SARA2; SARB. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 信号转导 结合蛋白 G蛋白信号 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Cow, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 22kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human SAR1B |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. |
| 产品介绍 | background: The protein encoded by this gene is a small GTPase that acts as a homodimer. The encoded protein is activated by the guanine nucleotide exchange factor PREB and is involved in protein transport from the endoplasmic reticulum to the Golgi. This protein is part of the COPII coat complex. Defects in this gene are a cause of chylomicron retention disease (CMRD), also known as Anderson disease (ANDD). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Mar 2010] Function: Involved in transport from the endoplasmic reticulum to the Golgi apparatus. Activated by the guanine nucleotide exchange factor PREB. Involved in the selection of the protein cargo and the assembly of the COPII coat complex. Subunit: Homodimer. Binds PREB. Part of the COPII coat complex. Binds to the cytoplasmic tails of target proteins in the endoplasmic reticulum (By similarity). Subcellular Location: Endoplasmic reticulum membrane. Golgi apparatus > Golgi stack membrane. Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region. Tissue Specificity: Expressed in many tissues including small intestine, liver, muscle and brain. DISEASE: Defects in SAR1B are the cause of chylomicron retention disease (CMRD) [MIM:246700]; also known as Anderson disease (ANDD). CMRD is an autosomal recessive disorder of severe fat malabsorption associated with failure to thrive in infancy. The condition is characterized by deficiency of fat-soluble vitamins, low blood cholesterol levels, and a selective absence of chylomicrons from blood. Affected individuals accumulate chylomicron-like particles in membrane-bound compartments of enterocytes, which contain large cytosolic lipid droplets. Similarity: Belongs to the small GTPase superfamily. SAR1 family. Database links: Entrez Gene: 51128 Human Entrez Gene: 416314 Chicken Entrez Gene: 515999 Cow Entrez Gene: 66397 Mouse Entrez Gene: 494017 Pig Entrez Gene: 287276 Rat Omim: 607690 Human SwissProt: Q3T0T7 Cow SwissProt: Q9Y6B6 Human SwissProt: Q9CQC9 Mouse SwissProt: Q5PYH3 Pig SwissProt: Q5HZY2 Rat Unigene: 432984 Human Unigene: 196592 Mouse Unigene: 36748 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
由该基因编码的蛋白质是一个小的GTPase，它充当同源二聚体。编码蛋白被鸟嘌呤核苷酸交换因子PREB激活，参与从内质网到高尔基体的蛋白质转运。这种蛋白质是COPII涂层复合物的一部分。该基因的缺陷是乳糜微粒滞留疾病（CMRD）的原因，也称为Anderson病（AND）。已经发现了两个编码相同蛋白的转录变体。〔RefSeq，2010〕