D-glyceric acid (DGA) is an intermediate of serine catabolism and of a minor pathway of fructose metabolism. The only known fate of DGA is phosphorylation to 3-phospho-D-glyceric acid (3PDGA) by cytosolic glycerate kinase (GLYCTK) (Gou et al. 2006). Defects in GLYCTK can cause D-glyceric aciduria (D-GA; MIM:220120), a rare inborn error of serine and fructose metabolism where DGA is excreted in large amounts in the urine. A variable phenotype is observed, ranging from severe mental retardation and death to milder speech delays and normal development (Van Schaftingen 1989, Sass et al. 2010).
Guo, JH, Hexige, S, Chen, L, Zhou, GJ, Wang, X, Jiang, JM, Kong, YH, Ji, GQ, Wu, CQ, Zhao, SY, Yu, L
Sass, JO, Fischer, K, Wang, R, Christensen, E, Scholl-Bürgi, S, Chang, R, Kapelari, K, Walter, M
Van Schaftingen, E
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