results the relative expression of mtdna copy number was 3.7 fold higher in nafld patients than healthy controls (p < 0.0001). the results remained significant after adjustment for age, bmi, and gender (p = 0.02). in addition, the mtdna copy number was 4.3 (p < 0.0001) and 3.2-fold (p < 0.0001) higher in nonalcoholic fatty liver (nafl) and non-alcoholic steatohepatitis (nash) patients than heal...

during two years study about mitochondrial disease (sep 1999-agu 2001), 15 cases of leigh syndrome (ls) were diagnosed, that consisted of 11 boys and 4 girls aged between 6 to 156 (mean: 40.5) months. most of the patients (46.7%) became symptomatic between 1-5 years of age. triggering factors were reported in 66.6% of the patients and 40% of them became symptomatic after infections. the most fr...

huntington disease (hd) is a genetically dominant condition caused by expanded cag repeats which code for glutamine in the hd gene product, huntingtin. huntingtin is expressed in almost all tissues, so abnormalities outside the brain can also be expected. involvement of nuclei and mitochondria in hd pathophysiology has been suggested. in fact, mitochondrial dysfunction is reported in brains of ...

oxidative stress and mitochondrial dysfunction are the main suggested mechanisms for neurodegenerative diseases. in this study, we have evaluated the effects of epicatechin (ec) on mitochondrial damage induced by homocycteine (hcy) using isolated rat hippocampus mitochondria in vivo . ec (50 mg/kg) was gavaged daily for a period of 10 days, starting 5 days prior to hcy (0.5 μmol/μl) intra hippo...

Multiple Sclerosis (MS) is a neurodegenerative and autoimmune disease that it’s molecular etiology and factors involving in its progression remains unknown. In this study for evaluation effect of mercuric on progression of MS we investigated the additive effect of mercuric sulfide on the brain mitochondrial dysfunction in experimental autoimmune encephalomyelitis (EAE) model of MS in C57BL/6 mi...

introduction: neurodegenerative diseases are progressive disorders that could impair neuronal functions and structures. oxidative stress and mitochondrial dysfunction are involved in the etiology of neurodegenerative diseases such as alzheimer’s disease, parkinson’s disease and etc. gemfibrozil is used as a therapeutic drug for hyperlipidemia. it has been shown that gemfibrozil is neuroprotecti...

Multiple Sclerosis (MS) is a neurodegenerative and autoimmune disease that it’s molecular etiology and factors involving in its progression remains unknown. In this study for evaluation effect of mercuric on progression of MS we investigated the additive effect of mercuric sulfide on the brain mitochondrial dysfunction in experimental autoimmune encephalomyelitis (EAE) model of MS in C57BL/6 mi...

objective: pompe disease is a rare neuromuscular genetic disorder and is classified into two forms of early and late-onset. over the past two decades, mitochondrial abnormalities have been recognized as an important contributor to an array of neuromuscular diseases. we therefore aimed to compare mitochondrial copy number and mitochondrial displacement-loop sequence variation in infantile and ad...