Suzuki S, Hinokio Y, Ohtomo M, Hirai M, Hirai A, Chiba M, Kasuga S, Satoh Y, Akai H, Toyota T
Third Department of Internal Medicine, Tohoku University School of Medicine, Sendai, Japan.
Diabetologia. 1998 May;41(5):584-8. doi: 10.1007/s001250050950.
The characteristic clinical features of diabetes mellitus with mitochondrial DNA (mtDNA) 3243(A-G) mutation are progressive insulin secretory defect, neurosensory deafness and maternal inheritance, referred to as maternally inherited diabetes mellitus and deafness (MIDD). A treatment for MIDD to improve insulin secretory defects and reduce deafness has not been established. The effects of coenzyme Q10 (CoQ10) treatment on insulin secretory response, hearing capacity and clinical symptoms of MIDD were investigated. 28 MIDD patients (CoQ10-DM), 7 mutant subjects with impaired glucose tolerance (IGT), and 15 mutant subjects with normal glucose tolerance (NGT) were treated daily with oral administration of 150 mg of CoQ10 for 3 years. Insulin secretory response, blood lactate after exercise, hearing capacity and other laboratory examinations were investigated every year. In the same way we evaluated 16 MIDD patients (control-DM), 5 mutant IGT and 5 mutant NGT subjects in yearly examinations. The insulin secretory response assessed by glucagon-induced C-peptide secretion and 24 h urinary C-peptide excretion after 3 years in the CoQ10-DM group was significantly higher than that in the control-DM group. CoQ10 therapy prevented progressive hearing loss and improved blood lactate after exercise in the MIDD patients. CoQ10 treatment did not affect the diabetic complications or other clinical symptoms of MIDD patients. CoQ10 treatment did not affect the insulin secretory capacity of the mutant IGT and NGT subjects. There were no side effects during therapy. This is the first report demonstrating the therapeutic usefulness of CoQ10 on MIDD.
线粒体DNA（mtDNA）3243（A-G）突变所致糖尿病的特征性临床特点为进行性胰岛素分泌缺陷、神经感觉性耳聋及母系遗传，称为母系遗传糖尿病伴耳聋（MIDD）。目前尚未确立改善MIDD胰岛素分泌缺陷及减轻耳聋的治疗方法。本研究调查了辅酶Q10（CoQ10）治疗对MIDD患者胰岛素分泌反应、听力及临床症状的影响。28例MIDD患者（CoQ10-DM组）、7例糖耐量受损（IGT）的突变受试者及15例糖耐量正常（NGT）的突变受试者每日口服150 mg CoQ10，共治疗3年。每年对胰岛素分泌反应、运动后血乳酸、听力及其他实验室检查项目进行调查。同样地，我们每年对16例MIDD患者（对照-DM组）、5例突变IGT受试者及5例突变NGT受试者进行评估。CoQ10-DM组治疗3年后，通过胰高血糖素诱导的C肽分泌及24小时尿C肽排泄评估的胰岛素分泌反应显著高于对照-DM组。CoQ10治疗可预防MIDD患者听力进行性下降，并改善运动后血乳酸水平。CoQ10治疗不影响MIDD患者的糖尿病并发症或其他临床症状。CoQ10治疗不影响突变IGT及NGT受试者的胰岛素分泌能力。治疗期间未出现副作用。这是首篇证明CoQ10对MIDD具有治疗作用的报告。
