Reactome | GLYCTK phosphorylates DGA to 3PDGA

GLYCTK phosphorylates DGA to 3PDGA

Stable Identifier

R-HSA-6799495

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol

Synonyms

(R)-glycerate + ATP => (2R)-3-phosphoglycerate + ADP + H(+)

ReviewStatus

5/5

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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) (Yu 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
20949620	D-glyceric aciduria is caused by genetic deficiency of D-glycerate kinase (GLYCTK) Sass, JO, Kapelari, K, Fischer, K, Wang, R, Scholl-Bürgi, S, Chang, R, Christensen, E, Walter, M	Hum. Mutat.	2010
16753811	Isolation and characterization of the human D-glyceric acidemia related glycerate kinase gene GLYCTK1 and its alternatively splicing variant GLYCTK2 Yu, L, Guo, JH, Zhao, SY, Wu, CQ, Wang, X, Hexige, S, Jiang, JM, Kong, YH, Chen, L, Ji, GQ, Zhou, GJ	DNA Seq.	2006
2537226	D-glycerate kinase deficiency as a cause of D-glyceric aciduria Van Schaftingen, E	FEBS Lett.	1989