Glycollic acid hyperoxaluria

Dietzen DJ, Wilhite TR, Kenagy DN, Milliner DS, Smith CH, Landt M Extraction of glyceric and glycolic acids from urine with tetrahydrofuran utility in detection of primary hyperoxaluria. Clin Chem 1997 43 1315-1320. 

Glyoxalate can be transaminated to glycine, reduced to glycolate, converted to a-hydroxy-/3-ketoadipate by reaction with a-ketoglutarate, or oxidized to oxalate and excreted in urine. The first three reactions require pyridoxal phosphate, NADH, and thiamine pyrophosphate, respectively. In humans, ascorbic acid (vitamin C) is a precursor of urinary oxalate (Chapter 38). Since calcium oxalate is poorly soluble in water, it can cause nephrolithiasis and nephrocalcinosis due to hyperoxaluria. 

Primary hyperoxaluria Type I (glycollic aciduria) Glycollic, oxalic and glyoxylic acids Cytosol glyoxylate 2-oxoglutarate carboligase 16.3.1... 

Fig. 16.4 Chromatogram of organic acids extracted using DEAE-Saphadex from the urine of a patient with primary hyperoxaluria type I, separated as their ethoxime and trimethylsilyl derivatives on 10 per cent OV-101 on HP Chromosorb W (80-KX) mesh) using temperature programming from 110°C to 285°C at 4°C min with a 5 min initial isothermal delay. Peak identifications are 1, glyoxylate 2, glycollate 3, oxalate 4, sulphate 5, phosphate 6, tetronates 7, citrate 8, undecanedioate (internalstandard) 9, Az-tetracosane (standard) 10, -hexaxosane (standard).

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