Reactive species
Human;Rat
Antibody type
Monoclonal antibodies
Immunogen
Purified recombinant human Mannose Phosphate Isomerase protein fragments expressed in E.coli.
Specificity
This antibody detects endogenous levels of Mannose Phosphate Isomerase and does not cross-react with related proteins.
Constitute
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
wb 1:1000 icc 1:300
Purification process
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
Other name
PMI1;CDG1B;FLJ39201;Mannose 6 phosphate isomerase;Mannose-6-phosphate isomerase; MANNOSEPHOSPHATE ISOMERASE;MGC94106;MPI;MPI_HUMAN;Phosphohexomutase;phosphomannose isomerase 1;Phosphomannose isomerase;PMI;PMI1.
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
catalytic activity:D-mannose 6-phosphate = D-fructose 6-phosphate.,cofactor:Binds 1 zinc ion per subunit.,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.,function:Involved in the synthesis of the GDP-mannose and dolichol-phosphate-mannose required for a number of critical mannosyl transfer reactions.,pathway:Nucleotide-sugar biosynthesis; GDP-D-mannose biosynthesis; alpha-D-mannose 1-phosphate from D-fructose 6-phosphate: step 1/2.,similarity:Belongs to the mannose-6-phosp
