Function
Phosphomannomutase (PMM) catalyzes the conversion of α-D-mannose 1-phosphate to D-mannose 6-phosphate. PMM participates in fructose and mannose metabolism.
Disease
Mutations in PMM2 cause carbohydrate-deficient glycoprotein syndrome type 1 (Jaeken syndrome)[1].
Structural highlights
The PMM structure contains a . It is known that in order for the substrate to bind, the cap must dissociate from the core domain and then re-associate to seal the active site from solvent molecules. The [2]. Water molecules are shown as red spheres.