An erythrocyte transketolase isoenzyme pattern associated with the Wernicke‐Korsakoff syndrome

Abstract

Two techniques were used to seek variants of human erythrocyte transketolase and to test for any association of the Wernicke-Korsakoff syndrome, a thiamin-deficiency disease, with a particular variant of this thiamin-dependent enzyme. Apparent Km values for the cofactor thiamin diphosphate were similar for patients and controls. However, isoelectric focussing separated erythrocyte transketolase into different isoenzymes, characterized by pI values in the range 6.6-9.2. Six distinct patterns of isoenzymes were found in 36 healthy control subjects. The isoenzyme pattern for 39 of 42 patients suffering from the Wernicke-Korsakoff syndrome was identical to a pattern found in only 8 of 36 control subjects, a highly significant association (P < 0.001). A variant transketolase and thiamin deficiency together evidently contribute to the pathogenesis of the brain damage of the Wernicke-Korsakoff syndrome by some mechanism independent of apparent Km values for thiamin diphosphate.