CoQ10 Effects on MDM Liver Fat Via FibroTouchI.

Mitochondrial diabetes mellitus (MDM) is a rare subtype of diabetes caused by mitochondrial dysfunction, often accompanied by hepatic steatosis. Coenzyme Q10 (CoQ10), a key electron carrier and antioxidant, may improve mitochondrial function and lipid metabolism. This prospective study aims to evaluate the effect of 12-week CoQ10 supplementation (300 mg/day) on liver fat content (assessed by FibroTouch CAP ) in MDM patients with m.3243A\>G mutation. An exploratory case-control design will compare baseline characteristics among MDM patients, type 1 diabetes patients, and healthy controls. This study is the first to explore the link between mitochondrial defects and hepatic fat accumulation specifically in MDM, and to test CoQ10 as a potential therapy, offering new insights for both MDM and MAFLD management.

1\. Mitochondrial Diabetes Group Inclusion Criteria: 1. Confirmed as a carrier of the mitochondrial DNA m.3243A\>G mutation through genetic testing; 2. Meeting the diagnostic criteria for diabetes (WHO 1999 diagnostic criteria); 3. Aged between 18 and 70 years; 4. Willing to participate and having signed the informed consent form. Exclusion Criteria: 1. Type 1 or type 2 diabetes (not caused by mitochondrial gene mutations); 2. Comorbid with viral hepatitis, drug-induced hepatitis, alcoholic liver disease, Wilson's disease, or other specific diseases that can lead to fatty liver; 3. Severe cardiac, pulmonary, or renal insufficiency; 4. Pregnant or lactating women; 5. Having used coenzyme Q10 or other mitochondrial-targeted drugs within the past 3 months; 6. Having contraindications to MRI examination; 7. Having experienced infection, surgery, or major trauma within the past 4 weeks. Withdrawal/Dropout Criteria: 1. The subject withdraws informed consent; 2. The occurrence of serious adverse events; 3. Loss to follow-up; 4. Medication adherence that continued participation in \< 80%; 5. The researcher believes the study is not in the best interest of the subject. 2. Type 1 Diabetes Control Group Inclusion Criteria: 1. Meeting the diagnostic criteria for type 1 diabetes, with positive islet autoantibodies (GAD, IA-2, ICA, etc.); 2. Aged between 18 and 70 years; 3. Willing to participate and having signed the informed consent form. Exclusion Criteria: Same as the exclusion criteria (1) to (7) for the mitochondrial diabetes group. 3. Healthy Adult Control Group Inclusion Criteria: 1. No history of diabetes, with fasting blood glucose \< 5.6 mmol/L and HbA1c \< 5.7%; 2. Aged between 18 and 70 years; 3. Willing to participate and having signed the informed consent form. Exclusion Criteria: Same as the exclusion criteria (1) to (7) for the mitochondrial diabetes group.