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It is expected that most mitochondrial disorders are caused by mutations in nuclear genes.
Treatment for MDS, like most mitochondrial disorders, has been limited to supportive therapies; however, understanding the pathomechanism of MDS enables the design of treatment strategies targeting either the cause of the disease or the downstream metabolic defects.
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Most pediatric mitochondrial disorders are caused by defects in nuclear genes and with an autosomal recessive inheritance [ 4].
Leber's hereditary optic neuropathy (LHON; MIM 535000) is the most common mitochondrial disorder affecting more than 1 14 000 males (Man et al., 2003).
Some of the most commonly recognized mitochondrial disorders are neurological conditions, heart problems and muscular dystrophy.
Its deficiency is the most frequently encountered in mitochondrial disorders.
Complex I deficiencies are the most common causes of mitochondrial disorders.
Clinical issue Deficiency of respiratory complex I is the most frequent causative factor underlying mitochondrial disorders.
Here, we have investigated OXPHOS regulation by the mt-sAC pathway in genetic models of COX deficiency, which is one of the most common causes of inherited mitochondrial disorders (Shoubridge, 2001).
Mitochondrial disorders are the most common group of metabolic disorders, with an estimated prevalence of 1 in 5,000 [ 1].
Mitochondrial disorders are the most common cause of inherited metabolic disease of adults and children, with no means of cure.
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