Reactome | MPI isomerises Fru6P to Man6P

MPI isomerises Fru6P to Man6P

Stable Identifier

R-HSA-532549

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

Fructose 6-phosphate isomerizes to Mannose 6-phosphate

Locations in the PathwayBrowser

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Metabolism of proteins (Homo sapiens)

- Post-translational protein modification (Homo sapiens)
  - Asparagine N-linked glycosylation (Homo sapiens)
    - Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein (Homo sapiens)
      - Synthesis of substrates in N-glycan biosythesis (Homo sapiens)
        
        Synthesis of GDP-mannose (Homo sapiens)
        
        MPI isomerises Fru6P to Man6P (Homo sapiens)

General

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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