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产品信息
PA5-64937
| 应用 | 建议稀释比 | 已发表文章 |
|---|---|---|
免疫印迹 (WB) |
0.04-0.4 µg/mL | - |
免疫细胞化学 (ICC/IF) |
0.25-2 µg/mL | - |
| 产品规格 | |
|---|---|
种属反应 |
Human |
宿主/亚型 |
Rabbit / IgG |
分类 |
Polyclonal |
类型 |
Antibody |
抗原 |
Recombinant Human GCS1 |
偶联物 |
Unconjugated |
形式 |
Liquid |
浓度 |
0.05 mg/mL |
纯化类型 |
Antigen affinity chromatography |
保存液 |
PBS, pH 7.2, with 40% glycerol |
内含物 |
0.02% sodium azide |
保存条件 |
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
RRID |
AB_2663261 |
产品详细信息
Immunogen sequence: LESHAEGFRER FEKTFQLKEK GLSSGEQVLG QAALSGLLGG IGYFYGQGLV LPDIGVEGSE QKVDPALFPP VPLFTAVPSR SFFPRGFLWD EGFHQLVVQR WDPSLTREAL GHWLGLLNAD GWIG
Highest antigen sequence identity to the following orthologs - mouse 89%, rat 88%.
靶标信息
GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and 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 include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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生物信息学
蛋白别名: glucosidase I; Mannosyl-oligosaccharide glucosidase; Processing A-glucosidase I
基因别名: CDG2B; CWH41; DER7; GCS1; MOGS
UniProt ID: (Human) Q13724
Entrez Gene ID: (Human) 7841
分子生物学功能:
glucosidase
