MPI isomerises Fru6P to Man6P

Stable Identifier
R-HSA-532549
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Synonyms
Fructose 6-phosphate isomerizes to Mannose 6-phosphate
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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
8307007 Purification, cDNA cloning and heterologous expression of human phosphomannose isomerase

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

Eur J Biochem 1994
10980531 Genomic organization of the human phosphomannose isomerase (MPI) gene and mutation analysis in patients with congenital disorders of glycosylation type Ib (CDG-Ib)

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

Hum Mutat 2000
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
mannose-6-phosphate isomerase activity of MPI [cytosol]
Physical Entity
Activity
Orthologous Events
Authored
Reviewed
Created