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2 Possible Causes for Secondary Decreases in Complex I and IV Activity

  • Acute Infantile Liver Failure-Multisystemic Involvement Syndrome

    In biliary atresia we detected variable mitochondrial alterations (low complex IV activity, decreased complex I and V on BN PAGE, low mtDNA copy number) in 20–50 % of patients[] […] result of a more complex cellular phenotype and can contribute to the hepatocellular dysfunction in various conditions.[] […] detection of mitochondrial abnormalities in a relatively large number of patients with a wide range of diagnoses raises the possibility that oxidative phosphorylation may be a secondary[]

  • Mitochondrial Myopathy and Sideroblastic Anemia

    complex I and IV activities with reduced complex III activity in a subset of patients [ 3 – 5, 7, 10 – 13 ].[] […] with some patients diagnosed in infancy with sideroblastic anemia and/or failure to thrive whereas other patients present in childhood or later with exercise intolerance secondary[] […] all patients described have demonstrated lactic acidemia, and although respiratory chain studies in fibroblasts may be normal, skeletal muscle biopsies typically display decreased[]

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