L-Glyceric acid is a mainly urinary metabolite accumulating in rare inherited metabolic disease L-glyceric aciduria. L-Glyceric acid can be used to diagnose primary hyperoxaluria type 2 (PH2). L-Glyceric acid excretion to distinguish PH1 from PH2[1][2].
Primary hyperoxaluria type 2 (PH2), also called L-glyceric aciduria. The metabolic defect is due to deficiencies of D-glycerate dehydrogenase and glyoxylate reductase, leading to excretion of L-Glyceric acid, and L-Glyceric acid is the cornerstone for the diagnosis of PH2[1][2].
[1]. Mohamed S Rashed, et al. Chiral liquid chromatography tandem mass spectrometry in the determination of the configuration of glyceric acid in urine of patients with D-glyceric and L-glyceric acidurias. Biomed Chromatogr. 2002 May;16(3):191-8. [2]. Bernd Hoppe, et al. A United States survey on diagnosis, treatment, and outcome of primary hyperoxaluria. Pediatr Nephrol. 2003 Oct;18(10):986-91.