Carnitine glycine

Treatment for nonketotic hyperglycinemia is less effective than that available for other aminoacidurias. There is no specific therapy. Exchange transfusion and dialysis usually do not alter the progressive neurological deterioration. Sodium benzoate has been administered in the hope that glycine would react with it to form hippuric acid, but this approach is not helpful. It may be that a combination of benzoate and carnitine therapy is more effective [28]. Similarly, the restriction of dietary protein... 

T Kanazu, T Yamaguchi. Comparison of in vitro carnitine and glycine conjugation with branched-side chain and cyclic side chain carboxylic acids in rats. Drug Metab Dispos 25(2) 149—153, 1997. 

Palmitoyl-DL-carnitine A/-Myristoyl-L-propyl-L-propyl-glycinate Saponins Concanavalin A... 

In propionic and methylmalonic aciduria, metronidazole, given orally, inhibits the production of propionic acid by gut bacteria. In isovaleric aciduria and methylcrotonyl-CoA carboxylase deficiency, glycine accompanied by carnitine supplementation inCTeases the elimination of toxic metabolites. In many severe conditions, empiric administration of substances that act as cofactors proves to be helpful, and this treatment option should not be neglected (Table 5.3) [19]. 

Fries MH, et til. Isovaleric acidemia response to a leucine load after three weeks of supplementation with glycine, L-camitine, and combined glycine-carnitine therapy. J Pediatr. 1996 129(3) 449-52. 

Carnitine, vitamia BjT a carrier of acetyl and acyl groups through the mitochondrial membrane (see Fatty add degradation). C. is characteristically present in muscle tissue, and has been isolated in crystalline form fi-om yeast. Mammals can synthesize C, but insects must obtain it in their diet, otherwise they are unable to complete metamorphosis. C is biosynthesized from lysine and y-butyrobetaine (Fig.) and degraded via glycine betaine and 2-methylcholine. 

Biosynthesis of carnitine. v-Butyrobetaine, 2-oxoglutarate dioxygenase (EC 1.14.11.1) occurs in liver, kidney and brain, whereas the other enzymes occur in most tissues. The aldolase cleaving 3-hydroxy-NMrimethyl-L-lysine may be identical with glycine hydroxymethyltransferase (EC 2.1.2.1). (W. A. Dunn etal. J. Biol. Chem. 259(1984) 10764-107701... 

Isovaleryl-CoA dehydrogenase (EC 1.3.99.10). Defective conversion of isovaleryl-CoA to methylcro-tonyl-CoA (see Leudne). Elevated isovalerate in plasma and urine also increased urinary isovaleryl-glydne, isovalerylcamitine and sometimes 3-hydro-xyisovalerate. Ketoacidotic crises, sometimes with fatal coma. Slight mental retardation in survivors. Treated with low leucine diet and supplements of glycine and/or carnitine to increase excretion of isovaler-yl conjugates. Peritoneal dialysis in crises. 

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