FITC标记的磷酸甘露糖异构酶抗体
产品名称： FITC标记的磷酸甘露糖异构酶抗体
英文名称： Anti-MPI/Mannose Phosphate Isomerase/FITC
产品编号： HZ-18654R-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-MPI/Mannose Phosphate Isomerase/FITC Conjugated antibody
FITC标记的磷酸甘露糖异构酶抗体
| 英文名称 | Anti-MPI/Mannose Phosphate Isomerase/FITC |
| 中文名称 | FITC标记的磷酸甘露糖异构酶抗体 |
| 别 名 | PMI1; CDG1B; FLJ39201; Mannose 6 phosphate isomerase; Mannose-6-phosphate isomerase; MANNOSEPHOSPHATE ISOMERASE; MGC94106; MPI; MPI_HUMAN; Phosphohexomutase; phosphomannose isomerase 1; Phosphomannose isomerase; PMI. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 免疫学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Cow, Horse, Sheep, |
| 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 | 46kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human MPI/Mannose Phosphate Isomerase |
| 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: Phosphomannose isomerase catalyzes the interconversion of fructose-6-phosphate and mannose-6-phosphate and plays a critical role in maintaining the supply of D-mannose derivatives, which are required for most glycosylation reactions. Mutations in the MPI gene were found in patients with carbohydrate-deficient glycoprotein syndrome, type Ib. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2014] Function: Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions. Subcellular Location: Cytoplasm. Tissue Specificity: Expressed in all tissues, but more abundant in heart, brain and skeletal muscle. DISEASE: Defects in MPI are the cause of congenital disorder of glycosylation type 1B (CDG1B) [MIM:602579]; also known as carbohydrate-deficient glycoprotein syndrome type Ib (CDGS1B). Congenital disorders of glycosylation are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1B is clinically characterized by protein-losing enteropathy. Similarity: Belongs to the mannose-6-phosphate isomerase type 1 family. Database links: Entrez Gene: 4351 Human Entrez Gene: 513586 Cow Entrez Gene: 110119 Mouse Entrez Gene: 300741 Rat Omim: 154550 Human SwissProt: Q3SZI0 Cow SwissProt: P34949 Human SwissProt: Q924M7 Mouse SwissProt: Q68FX1 Rat Unigene: 75694 Human Unigene: 247218 Mouse Unigene: 44246 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
磷酸腺苷酶异构酶催化果糖-6-磷酸和甘露糖-6-磷酸的相互转化，在维持D-甘露糖衍生物的供应中起关键作用，D-甘露糖衍生物是大多数糖基化反应所必需的。在Ib型糖缺乏型糖蛋白综合征患者中发现了MPI基因突变。选择性剪接导致多个转录体变异。[ RefSeq，2014月1日]