D-Glucose Metabolism in Lymphocytes of Patients with Mitochondrial Point Mutation of the tRNALeu(UUR) Gene

D-Glucose metabolism was examined in the lymphocytes of six subjects with the mitochondrial tRNA^Leu(UUR) gene mutation responsible for the maternally inherited diabetes and deafness MIDD syndrome and compared with control subjects. No significant difference in D-[1-¹⁴C]glucose, D-[2-¹⁴C]glucose, or D-[6-¹⁴C]glucose oxidation, as well as D-[5-³H]glucose utilization, was observed between the two groups of subjects. These negative findings stress the view that impaired D-glucose metabolism, such as presumably is occurring in the β-cells of patients with the MIDD syndrome, does not represent a universal feature found in all cell types of these patients.Copyright 1995, 1999 Academic Press, Inc.