4ufj | pdb_00004ufj

Mouse Galactocerebrosidase complexed with iso-galacto-fagomine lactam IGL

spinqualitylabelsall models 4ufj, resolution 2.20Å Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image   Print3D Structural highlights 4ufj is a 1 chain structure. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance. Ligands: CA, IF7, NAG Related: 4ufh, 4ufi, 4ufk, 4ufl, 4ufm Activity: Galactosylceramidase, with EC number 3.2.1.46 Resources: FirstGlance, OCA, RCSB, PDBsum Disease [GALC_MOUSE] Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. Function [GALC_MOUSE] Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.

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