6g7o

Crystal structure of human alkaline ceramidase 3 (ACER3) at 2.7 Angstrom resolution

spinqualitylabelsall models 6g7o, resolution 2.70Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 6g7o is a 1 chain structure with sequence from Escherichia coli and Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Method: X-ray diffraction, Resolution 2.7Å Ligands: CA, MG, NA, OLB, SO4, ZN Resources: FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT Disease ACER3_HUMAN Alkaline ceramidase 3 deficiency. Function C562_ECOLX Electron-transport protein of unknown function.ACER3_HUMAN Hydrolyzes only phytoceramide into phytosphingosine and free fatty acid. Does not have reverse activity. Publication Abstract from PubMed Alkaline ceramidases (ACERs) are a class of poorly understood transmembrane enzymes controlling the homeostasis of ceramides. They are implicated in human pathophysiology, including progressive leukodystrophy, colon cancer as well as acute myeloid leukemia. We report here the crystal structure of the human ACER type 3 (ACER3). Together with computational studies, the structure reveals that ACER3 is an intramembrane enzyme with a seven transmembrane domain architecture and a catalytic Zn(2+) binding site in its core, similar to adiponectin receptors. Interestingly, we uncover a Ca(2+) binding site physically and functionally connected to the Zn(2+) providing a structural explanation for the known regulatory role of Ca(2+) on ACER3 enzymatic activity and for the loss of function in E33G-ACER3 mutant found in leukodystrophic patients. Structure of a human intramembrane ceramidase explains enzymatic dysfunction found in leukodystrophy.,Vasiliauskaite-Brooks I, Healey RD, Rochaix P, Saint-Paul J, Sounier R, Grison C, Waltrich-Augusto T, Fortier M, Hoh F, Saied EM, Arenz C, Basu S, Leyrat C, Granier S Nat Commun. 2018 Dec 21;9(1):5437. doi: 10.1038/s41467-018-07864-w. PMID:30575723 From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine. See Also Ceramidase 3D PDB structures

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