Malaisse W J, Pueyo M E, Nadi A B, Malaisse-Lagae F, Froguel P, Velho G
Laboratory of Experimental Medicine, Brussels Free University, Belgium.
Biochem Mol Med. 1995 Apr;54(2):91-5. doi: 10.1006/bmme.1995.1013.
D-Glucose metabolism was examined in the lymphocytes of six subjects with the mitochondrial tRNALeu(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 beta-cells of patients with the MIDD syndrome, does not represent a universal feature found in all cell types of these patients.
在六名患有因线粒体tRNALeu(UUR)基因突变导致母系遗传糖尿病和耳聋的MIDD综合征患者的淋巴细胞中检测了D-葡萄糖代谢，并与对照受试者进行了比较。两组受试者在D-[1-14C]葡萄糖、D-[2-14C]葡萄糖或D-[6-14C]葡萄糖氧化以及D-[5-3H]葡萄糖利用方面均未观察到显著差异。这些阴性结果强调了这样一种观点，即D-葡萄糖代谢受损，比如可能发生在MIDD综合征患者的β细胞中，并不代表在这些患者的所有细胞类型中都存在的普遍特征。
