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产品中心-北京博奥森生物技术有限公司
Recombinant Human MOGS Protein, N-His (bs-105596P)  
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20ug/980.00元
50ug/1880.00元
100ug/2680.00元
大包装/询价
产品编号 bs-105596P
英文名称 Recombinant Human MOGS Protein, N-His
中文名称
别    名 GCS1; MOGS; Processing A-glucosidase I; Mannosyl-oligosaccharide glucosidase  
克 隆 号
理论分子量 16.14 kDa
检测分子量
性    状 Lyophilized
纯    度 >90% as determined by SDS-PAGE.
缓 冲 液 Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
保存条件 Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍 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.

SWISS:
Q13724

Gene ID:
7841

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