Duran 1 Organic Acids

Mitochondria from body wall muscle and probably the pharynx lack a functional TCA cycle and their novel anaerobic pathways rely on reduced organic acids as terminal electron acceptors, instead of oxygen (Saz, 1971 Ma et al, 1993 Duran et al, 1998). Malate and pyruvate are oxidized intramitochondrially by malic enzyme and the pyruvate dehydrogenase complex, respectively, and excess reducing power in the form of NADH drives Complex II and [3-oxidation in the direction opposite to that observed in aerobic organelles (Kita, 1992 Duran et al, 1993 Ma et al,... 

Hoffman GP. Organic acid analysis. In Blau N, Duran M, Blaskovics ME (eds) Physicians Guide to the Laboratory Diagnosis of Metabolic Diseases. Chapman Hall, London, pp 31-49... 

Arroyo Lopez, F.N., Duran Quintana, M.C., and Garrido, F.A. 2006. Microbial evolution during storage of seasoned olives prepared with organic acids with potassium sorbate, sodium benzoate, and ozone used as preservatives. Journal of Food Protection 69 1354—1364. 

Kamerling, J.P., Duran, M., Bruinvis, L., Ketting, D., Wadman, S.K., deGroot, C.J. and Hommes, F.A. (1977), (2-Ethoxyethoxy)acetic acid An unusual compound found in the gas chromatographic analysis of urinary organic acids. Clin. Chim. Acta, 77,397. 

Fig. 10.19 Chromatogram of organic acids extracted using ethyl acetate from the urine of a patient with 3-hydroxy-3-methylglutaric aciduria and separated as their trimethylsilyl derivatives on 5 per cent SE 52 on Chromosorb W (AW-DMCS, 100-120 mesh) using temperature programming from 75°C to 220°C at 2°C min with initial and final isothermal delays of 10 min. Peak identifications are 1, 3-hydroxyisovalerate 2, hydroxycaproate isomer 3, glutarate 4, 3-methylglutarate 5 and 6, 3-methyl-glutaconate peaks 7, adipate 8, 4-phenylbutyrate (internal standard) 9, 3-hydroxy-3-methylglutarate 10, ascorbate. (Redrawn with modifications from Duran ct. a/., 1978)...

Table 10.3 Organic acid concentrations in urine from a patient with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (after Duran 1978a Schutgens era/., 1978).

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