GALM catalyzes the interconversion of alpha- and beta-D-hexo...

created [InstanceEdit:9931849] Rothfels, Karen, 2024-12-13
dbId 9931852
displayName GALM catalyzes the interconversion of alpha- and beta-D-hexo...
modified [InstanceEdit:9931936] Rothfels, Karen, 2024-12-13
schemaClass Summation
text GALM catalyzes the interconversion of alpha- and beta-D-hexose sugars, with preference for galactose over glucose (Timson and Reese, 2003). Gel filtration and x-ray crystallography suggest that human GALM is monomeric like the E. coli enzyme (Timson and Reese, 2003; Thoden et al, 2004). Residues D307 and J176 are critical for enzymatic activity and are conserved across known galactose epimerases (Timson and Reese, 2003; Thoden et al, 2004). Mutations in GALM have been identified in cases of galactosemia, characterized by accumulation of unmetabolized galactose and leading to cataracts and in severe cases to kidney, liver and brain damage. GALM-mediated conversion of beta-Galactose to alpha-galactose is required prior to aGAL phosphorylation by galactokinase (GALK), the first committed step of galactose catabolism (Ai et al, 1995; Timson and Reese, 2003).
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