Reactome | PMM1,2 isomerise Man6P to Man1P

PMM1,2 isomerise Man6P to Man1P

Stable Identifier

R-HSA-446201

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

Mannose 6-phosphate isomerises to Mannose 1-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)
        
        PMM1,2 isomerise Man6P to Man1P (Homo sapiens)

General

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Phosphomannomutases 1 and 2 (PMM1 and PMM2) catalyse the isomerisation of mannose 6-phosphate (Man6P) to mannose 1-phosphate (Man1P) in the cytosol of cells (Wada & Sakamoto 1997, Matthijs et al. 1997). Mutations in the PMM2 gene are one of the causes of Jaeken syndrome. a disease of glycosylation, type CDGIa. (Matthijs et al. 1997b).

Literature References

PubMed ID	Title	Journal	Year
9119384	Isolation of the human phosphomannomutase gene (PMM1) and assignment to chromosome 22q13 Sakamoto, M, Wada, Y	Genomics	1997
9140401	Mutations in PMM2, a phosphomannomutase gene on chromosome 16p13, in carbohydrate-deficient glycoprotein type I syndrome (Jaeken syndrome) Cassiman, JJ, Matthijs, G, Veiga-da-Cunha, M, Schollen, E, Jaeken, J, Van Schaftingen, E, Pardon, E	Nat Genet	1997
9070917	PMM (PMM1), the human homologue of SEC53 or yeast phosphomannomutase, is localized on chromosome 22q13 Cassiman, JJ, Pirard, M, Matthijs, G, Schollen, E, Van Schaftingen, E, Budarf, ML	Genomics	1997