Glucocerebrosidases catalyze a transgalactosylation reaction that yields a newly-identified brain sterol metabolite, galactosylated cholesterol
Hisako Akiyama, Mitsuko Ide, Yasuko Nagatsuka, Tomoko Sayano, Etsuro Nakanishi, Norihito Uemura, Kohei Yuyama, Yoshiki Yamaguchi, Hiroyuki Kamiguchi, Ryōsuke Takahashi, Johannes M. F. G. Aerts, Peter Greimel, Yoshio Hirabayashi
Abstract
enzyme assays confirmed that GBA1 and GBA2 have transgalactosylation activity to transfer the galactose residue from GalCer to cholesterol to form β-GalChol. This is the first report of the existence of β-GalChol in vertebrates and how β-GlcChol and β-GalChol are formed in the brain.
Topics & Concepts
CeramideBiochemistrySterolMetaboliteEnzymeGlucocerebrosidaseGlucocerebrosideGlycolipidResidue (chemistry)BiologyBiosynthesisChemistryMetabolismCholesterolApoptosisLysosomal Storage Disorders ResearchSphingolipid Metabolism and SignalingLipid Membrane Structure and Behavior