Effect of various agents on adenosine triphosphate synthesis in mitochondrial complex I deficiency.
case_report · Level V
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Abstract
The effect of agents commonly used in the therapy of mitochondrial complex I deficiency was examined in fibroblasts from a patient. Marked improvement was observed with riboflavin, which nearly normalized the adenosine triphosphate production. The study of adenosine triphosphate production rate in fibroblasts may improve decision-making in treatment design of patients with respiratory chain defects.
Medical subject headings
- Adenosine Triphosphate
- Fibroblasts
- Leigh Disease
- Photosensitizing Agents
- Riboflavin