MPI isomerises Fru6P to Man6P

Stable Identifier
R-HSA-532549
Type
Reaction
Species
Homo sapiens
Compartment
Synonyms
Fructose 6-phosphate isomerizes to Mannose 6-phosphate
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

Mannose-6-phosphate isomerase (MPI) isomerises fructose 6-phosphate (Fru6P) to mannose 6-phosphate (Man6P) (Proudfoot et al. 1994). Defects in this gene are associated with congenital disorder of glycosylation type 1B (CDG1B). Oral administration of mannose is an efficient therapy against this defect (Schollen et al. 2000).

Literature References
PubMed ID Title Journal Year
10980531 Genomic organization of the human phosphomannose isomerase (MPI) gene and mutation analysis in patients with congenital disorders of glycosylation type Ib (CDG-Ib)

Freeze, H, Huijmans, JG, Schollen, E, Patterson, M, Pronicka, E, Babovic-Vuksanovic, D, Van Diggelen, OP, Matthijs, G, Adamowicz, M, Dorland, L, Marquardt, T, Winchester, B, Imtiaz, F, de Koning, TJ

Hum Mutat 2000
8307007 Purification, cDNA cloning and heterologous expression of human phosphomannose isomerase

Proudfoot, AE, Wells, TN, Payton, MA, Turcatti, G, Smith, DJ

Eur J Biochem 1994
Participants
Participates
Catalyst Activity

mannose-6-phosphate isomerase activity of MPI [cytosol]

Orthologous Events
Authored
Reviewed
Created
Cite Us!