6ddt | pdb_00006ddt

mouse beta-mannosidase (MANBA)

spinqualitylabelsall models 6ddt, resolution 2.10Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 6ddt is a 1 chain structure with sequence from Lk3 transgenic mice. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Ligands: CA, EDO, FUC, NAG Gene: Manba, Bmn (LK3 transgenic mice) Activity: Beta-mannosidase, with EC number 3.2.1.25 Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Function [MANBA_MOUSE] Exoglycosidase that cleaves the single beta-linked mannose residue from the non-reducing end of all N-linked glycoprotein oligosaccharides.[UniProtKB:O00462] Publication Abstract from PubMed beta-Mannosidase is a lysosomal enzyme from the glycosyl hydrolase family 2 that cleaves the single beta(1-4)-linked mannose at the nonreducing end of N-glycosylated proteins, and plays an important role in the polysaccharide degradation pathway. Mutations in the MANBA gene, which encodes the beta-mannosidase, can lead to the lysosomal storage disease beta-mannosidosis, as well as nystagmus, an eye condition characterized by involuntary eye movements. Here, we present the first structures of a mammalian beta-mannosidase in both the apo- and mannose-bound forms. The structure is similar to previously determined beta-mannosidase structures with regard to domain organization and fold, however, there are important differences that underlie substrate specificity between species. Additionally, in contrast to most other ligand-bound beta-mannosidases from bacterial and fungal sources where bound sugars were in a boat-like conformation, we find the mannose in the chair conformation. Evaluation of known disease mutations in the MANBA gene provides insight into their impact on disease phenotypes. Together, these results will be important for the design of therapeutics for treating diseases caused by beta-mannosidase deficiency. DATABASE: Structural data are available in the Protein Data Bank under the accession numbers 6DDT and 6DDU. The structure of mammalian beta-mannosidase provides insight into beta-mannosidosis and nystagmus.,Gytz H, Liang J, Liang Y, Gorelik A, Illes K, Nagar B FEBS J. 2018 Dec 15. doi: 10.1111/febs.14731. PMID:30552791 From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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