234 top medical experts on Mitochondrial complex I deficiency across 29 countries and 10 U.S. states, including 53 MDs (Physicians). This is based on an objective analysis of their Scientific Publications, Clinical Trials, Medicare, and NIH Grants.

  1. Mitochondrial complex I deficiency: Mitochondrial complex I deficiency is a type of mitochondrial disease . Mitochondria are specialized compartments in cells that create more than 90% of the energy needed by the body. In mitochondrial diseases, the mitochondria don't work correctly resulting in less energy in the cell, cell injury and cell death. Complex I is the first step in a chain reaction in mitochondria leading to energy production. Signs and symptoms of complex I deficiency vary widely in nature and severity, ranging from lethal neonatal disease to adult-onset neurodegenerative disorders. Features may include macrocephaly (large head) with progressive leukodystrophy , encephalopathy , hypertrophic cardiomyopathy , myopathy , liver disease, Leigh syndrome , Leber hereditary optic neuropathy , and some forms of Parkinson disease . The disease is caused by mutations in any of many genes and the inheritance pattern depends on the responsible gene. Treatment is only helpful in some case but may include metabolic therapies such as riboflavin , thiamine , biotin , co-enzyme Q10 , carnitine , and a ketogenic diet (a special high-fat, low-carbohydrate diet).
  2. Clinical guidelines are the recommended starting point to understand initial steps and current protocols in any disease or procedure:
  3. Support Organizations
  4. Synonyms: deficiency of Mitochondrial NADH dehydrogenase component of complex I,  NADH coenzyme q reductase deficiency,  NADH:Q(1) Oxidoreductase deficiency,  Nadh-Coenzyme Q Reductase Deficiency


  

        

                    


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