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-Ketoacids stimulate renal cysteine conjugate -lyase activity and potentiate the cytotoxicity of S-(1,2-dichlorovinyl)-L-cysteine

In a preferred embodiment of the invention, the CNS pathology is one of a peripheral disorder with a CNS component, such as septic shock, brain metastasis, hepatic encephalopathy, (diabetic) hypertension, diabetic (micro)angiopathy, sleeping sickness, Whipple disease, Duchenne muscular dystrophy (DMD), aspartylglucosaminuria, cholesterol ester storage disease, Wolman disease, cystinosis, Danon disease, Fabry disease, Farber lipogranulomatosis, Farber disease, fucosidosis, galactosialidosis types I/II, Gaucher disease types I/II/III, Gaucher disease, globoid cell leuco dystrophy, Krabbe disease, glycogen storage disease II, Pompe disease, GMl- gangliosidosis types 1/11/1 II, GM2-gangliosidosis type I, Tay Sachs disease, GM2- gangliosidosis type II, Sandhoff disease, GM2-gangliosidosis, alpha-mannosidosis types 1/1 1 , mannosidosis, metachromatic leucodystrophy, mucolipidosis type I, sialidosis types 1/11 mucolipidosis types l l/III 1-cell disease, mucolipidosis type UIC pseudo-Hurler poly dystrophy, mucopolysaccharidosis type I, mucopolysaccharidosis type II, Hunter syndrome, mucopolysaccharidosis type IIIA, Sanflippo syndrome, mucopolysaccharidosis type IHB, mucopolysaccharidosis type IHC, mucopolysaccharidosis type HID, mucopolysaccharidosis type IVA, Morquio syndrome, mucopolysaccharidosis type IVB Morquio syndrome, mucopolysaccharidosis type VI, mucopolysaccharidosis type VII, Sly syndrome, mucopolysaccharidosis type IX, multiple sulphatase deficiency, neuronal ceroid lipofuscinosis, CLNl Batten disease, Niemann-Pick disease types A/B, Niemann-Pick disease, Niemann-Pick disease type Cl, Niemann-Pick disease type C2 , pycnodysostosis, Schindler disease types VII, Schindler disease, sialic acid storage disease, and (pre)eclampsia

66 , 67 , 68 Moreover, just as in the case of G6PD A , many of the different biochemical variants have turned out to have the same molecular defect as G6PD Mediterranean (Table 28.1)