FITC标记的β-葡萄糖苷酶1抗体
产品名称： FITC标记的β-葡萄糖苷酶1抗体
英文名称： Anti-GCS1/FITC
产品编号： HZ-13322R-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-GCS1/FITC Conjugated antibody
FITC标记的β-葡萄糖苷酶1抗体
| 英文名称 | Anti-GCS1/FITC |
| 中文名称 | FITC标记的β-葡萄糖苷酶1抗体 |
| 别 名 | EC 3.2.1.106; glucosidase I; Mannosyl oligosaccharide glucosidase; Mannosyl-oligosaccharide glucosidase; Mogs; MOGS_HUMAN; Processing A glucosidase I; Processing A-glucosidase I. |
| 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 细胞类型标志物 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, |
| 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 | 92kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GCS1 |
| 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: Glycosylation of asparagine residues in Asn-X-Ser/Thr motifs in proteins commonly occur in the lumen of the endoplasmic reticulum (ER). Glucosidase I catalyzes the first step in the N-linked oligosaccharide processing pathway. It specifically removes the distal alpha 1,2-linked glucose residue from the Glc3-Man9-GlcNAc2 oligosaccharide precursor. Glucosidase I contains a short cytosolic tail, a single pass transmembrane domain and a large C-terminal catalytic domain located on the luminal side of the ER. Mutations in the gene encoding Glucosidase I result in the congenital disorder glycosylation (CDG-IIb), which is characterized by generalized hypotonia, dysmorphic features, hepatomegaly, hypoventilation, feeding problems, seizures and death. Two point mutations in the Glucosidase I gene have been identified and result in amino acid substitutions, namely Arg486Thr and Phe652Leu, that affect polypeptide folding and active site formation. Function: Cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Subcellular Location: Endoplasmic reticulum membrane. DISEASE: Defects in MOGS are the cause of type IIb congenital disorder of glycosylation (CDGIIb) [MIM:606056]; also known as glucosidase I deficiency. CDGIIb is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms of the infant included hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course was progressive and the infant did not survive more than a few months. Similarity: Belongs to the glycosyl hydrolase 63 family. Database links: UniProtKB/Swiss-Prot: Q13724.5 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic application |
天冬酰胺残基在蛋白中的Asn-X-Ser/Thr基序中的糖基化通常发生在内质网(ER)的腔内。葡萄糖苷酶I催化N-链寡糖加工途径中的第一步。它从GLC3-MAN-GLCNAC2寡糖前体中特异性地除去远端α1，2连接的葡萄糖残基。葡萄糖苷酶I包括位于ER管腔侧的短胞质尾、单程跨膜结构域和大的C端催化结构域。编码葡萄糖苷酶I的基因突变导致先天性糖基化障碍(CDG-IIb)，其特征为全身性低血压、形态异常、肝肿大、通气不足、喂养困难、癫痫发作和死亡。葡萄糖苷酶I基因的两个点突变已被鉴定，并导致氨基酸取代，即Arg486Thr和Pe652Leu，它们影响多肽折叠和活性位点的形成。