产品货号 :  mlR18654

英文名称 :  MPI/Mannose Phosphate Isomerase

中文名称 :  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.  

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 :  ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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.

分 子 量 :  46kDa

细胞定位 :  细胞浆

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human MPI/Mannose Phosphate Isomerase:61-160/423

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.

PubMed :  PubMed

产品介绍 :  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.

SWISS:

P34949

Gene ID:

4351

Important Note:

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.