Anti-MPI Picoband® Antibody (monoclonal, 5G5) Fluoro647 Conjugated

Mannose-6 phosphate isomerase (MPI), alternately phosphomannose isomerase (PMI), is an enzyme which facilitates the interconversion of fructose 6-phosphate (F6P) and mannose-6-phosphate (M6P) . It also 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. This MPI gene is mapped to 15q24.1.

E. coli-derived human MPI recombinant protein (Position: A2-K99) . Human MPI shares 88.8% and 86.7% amino acid (aa) sequence identity with mouse and rat MPI, respectively.

Channels, Energy Metabolism, Energy Transfer Pathways, Metabolic Signaling Pathways, Metabolism, Pathways and Processes, Plasma Membrane, Signal Transduction

1. Jaeken, J., Matthijs, G., Saudubray, J.-M., Dionisi-Vici, C., Bertini, E., de Lonlay, P., Henri, H., Carchon, H., Schollen, E., Van Schaftingen, E. Phosphomannose isomerase deficiency: a carbohydrate-deficient glycoprotein syndrome with hepatic-intestinal presentation. (Letter) Am. J. Hum. Genet. 62: 1535-1539, 1998. 2. Schollen, E., Dorland, L., de Koning, T. J., Van Diggelen, O. P., Huijmans, J. G. M., Marquardt, T., Babovic-Vuksanovic, D., Patterson, M., Imtiaz, F., Winchester, B., Adamowicz, M., Pronicka, E., Freeze, H., Matthijs, G. Genomic organization of the human phosphomannose isomerase (MPI) gene and mutation analysis in patients with congenital disorders of glycosylation type Ib (CDG-Ib) . Hum. Mutat. 16: 247-252, 2000.