D-2-Hydroxyglutarate

D-2-Hydroxyglutaric 2-Hydroxyglutaric, 2-oxoglutaric, succinic and glycollic acids Not known (Chapter 16)... 

Holmes, E. Foxall, P.J.D. Spraul, M. Farrant, R.D. Nicholson, J.K. Lindon, J.C. 750 MHz H-1 NMR Spectroscopy Characterisation of the Complex Metabolic Pattern of Urine from Patients with Inborn Errors of Metabolism 2-Hydroxyglutaric Aciduria and Maple Syrup Urine Disease, . 7. Pharm. Biomed. Anal. 15(11), 1647-1659 (1997). 

Hydroxyglutaric (HG) acid is an optically active organic acid. With respect to 2-HG acid two distinct disorders with very different phenotypes are known in one D-2-HG acid accumulates and in the other it is the L-form. Therefore it is important to determine the configuration of accumulating 2-HG acid. The separation of D- and L-2-HG acid requires the use of chiral GC- and/or HPLC-columns or the preparation of chiral derivatives using D-2-butanol [18]. 

A few patients with combined D-2- and L-2-hydroxyglutaric aciduria have been reported. Whether these patients represent another clinical and/... 

Fig. 8.1. Pathways of 2-ketoglutarate, fumarate, malonate and N-acetylaspartate metabolism and proposed pathways of D- and L-2-hydroxyglutarate metabolism. 8.1, 2-Ketoglutarate dehydrogenase complex (El or E2) 8.2, fumarase 8.3, malonyl-CoA decarboxylase 8.6, aspartoacylase...

Gibson KM, ten Brink HJ, Schor DSM, Kok RM, Bootsma AH, Hoffmann GF, Jakobs C (1993) Stable isotope dilution analysis of D- and L-2-hydroxyglutaric acid application to the detection and prenatal diagnosis of D- and L-2-hydroxyglutaric acidemias. Pediatr Res 34 277-280... 

Muntau AC, Roschinger W, Merkenschlager A, van der Knaap MS, Jakobs C, Duran M, Hoffmann GF, Rosher AA. Combined D-2- and L-2-hydroxyglutaric aciduria with neonatal onset encephalopathy A third biochemical variant of 2-hydroxyglutaric aciduria Neuropediatrics 2000 31 137-140... 

Recently, inherited metabolic diseases have been described in which a particular enantiomeric form of a metabolite is exclusively excreted [42]. Typical examples are D(-l-)-glyceric aciduria [43] and L-2-hydroxyglutaric aciduria [44], Using conventional GC-MS for urinary organic acid screening, increased amounts of these... 

Deoxy-D-manno-octulosonate aldolase, which facilitates the reaction of pyruvate with D-arabinose to form 3-deoxy-D-manno-octulosonate, an important component of bacterial cell walls. (5) 2-Oxo-4-hydroxyglutar-ate aldolase, which converts 2-oxo-4-hydroxyglutarate to pyruvate and glyoxylate. (6) Deoxyribose-phosphate aldolase, which converts 2-deoxy-D-ribose 5-phosphate... 

Other pyruvate- and phosphoenolpyruvate-dependent aldolases have been isolated and purified, but have not yet been extensively investigated for synthetic use. Those showing promise for future applications include, 3-deoxy-D-arabino-2-heptulosonic acid 7-phosphate (DAHP) synthetase (EC 4.1.2.15), 2-keto-4-hydroxyglutarate (KHG) aldolase (EC 4.1.2.31), and 2-keto-3-deoxy-D-gluconate (KDG) aldolase (EC 4.1.2.20). DAHP synthetase has been used... 

---