GLYCTK phosphorylates DGA to 3PDGA

Stable Identifier
R-HSA-6799495
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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).

Literature References
PubMed ID Title Journal Year
16753811 Isolation and characterization of the human D-glyceric acidemia related glycerate kinase gene GLYCTK1 and its alternatively splicing variant GLYCTK2

Guo, JH, Hexige, S, Chen, L, Zhou, GJ, Wang, X, Jiang, JM, Kong, YH, Ji, GQ, Wu, CQ, Zhao, SY, Yu, L

DNA Seq. 2006
20949620 D-glyceric aciduria is caused by genetic deficiency of D-glycerate kinase (GLYCTK)

Sass, JO, Fischer, K, Wang, R, Christensen, E, Scholl-B├╝rgi, S, Chang, R, Kapelari, K, Walter, M

Hum. Mutat. 2010
2537226 D-glycerate kinase deficiency as a cause of D-glyceric aciduria

Van Schaftingen, E

FEBS Lett. 1989
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
glycerate kinase activity of GLYCTK [cytosol]
Physical Entity
Activity
Orthologous Events
Cross References
Rhea
Authored
Reviewed
Created