磷酸甘露糖异构酶抗体
规格：1mg/1ml
英文名： MPI/Mannose Phosphate Isomerase
别名： PMI1; CDG1B; FLJ39201; Mannose 6 phosphate isomerase; Mannose-6-phosphate isomerase; MANNOSEPHOSPHATE ISOMERASE; MGC94106; MPI; MPI_HUMAN; Phosphohexomutase; phosphomannose isomerase 1; Phosphomannose
分子量： 46kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MPI/Mann
交叉反应：Human, Mouse, Rat, Pig, Cow, Horse, Sheep,
细胞定位：细胞浆
磷酸甘露糖异构酶抗体产品介绍：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磷酸甘露糖异构酶抗体 (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. Gene ID: 4351 Database links: Entrez Gene: 4351 Human Entrez Gene: 513586 Cow Entrez Gene: 磷酸甘露糖异构酶抗体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.
磷酸甘露糖异构酶抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid