| 產(chǎn)品編號 | bs-13322R |
| 英文名稱 | Rabbit Anti-GCS1 antibody |
| 中文名稱 | β-葡萄糖苷酶1抗體 |
| 別 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. |
| 研究領(lǐng)域 | 細胞生物 細胞類型標(biāo)志物 |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | (predicted: Human,Mouse,Rat,Dog) |
| 產(chǎn)品應(yīng)用 | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 92kDa |
| 細胞定位 | 細胞漿 細胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GCS1: 51-150/837 |
| 亞 型 | |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
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. SWISS: Q13724 Gene ID: 7841 Database links: Entrez Gene: 7841 Human Omim: 601336 Human SwissProt: Q13724 Human Unigene: 516119 Human |
