Phosphomannomutase

From Proteopedia

spinqualitylabelsall models Human phosphomannomutase 1 complex with α-D-mannose-1-phosphate and Mg+2 ion (green) (PDB entry 2fue) Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image 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.

3D Structures of phosphomannomutase

Updated on 28-August-2023

References

1. ↑ Matthijs G, Schollen E, Pardon E, Veiga-Da-Cunha M, Jaeken J, Cassiman JJ, Van Schaftingen E. Mutations in PMM2, a phosphomannomutase gene on chromosome 16p13, in carbohydrate-deficient glycoprotein type I syndrome (Jaeken syndrome). Nat Genet. 1997 May;16(1):88-92. PMID:9140401 doi:10.1038/ng0597-88
2. ↑ Silvaggi NR, Zhang C, Lu Z, Dai J, Dunaway-Mariano D, Allen KN. The X-ray crystal structures of human alpha-phosphomannomutase 1 reveal the structural basis of congenital disorder of glycosylation type 1a. J Biol Chem. 2006 May 26;281(21):14918-26. Epub 2006 Mar 15. PMID:16540464 doi:http://dx.doi.org/10.1074/jbc.M601505200