Effect of various agents on adenosine triphosphate synthesis in mitochondrial complex I deficiency.
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.
Bar-Meir
M
M
Metabolic Disease Unit, Shaare-Zedek Medical Center, Jerusalem, Israel.
Elpeleg
O N
ON
Saada
A
A
D000255:Adenosine Triphosphate / Q000096:biosynthesis*; D005347:Fibroblasts / Q000187:drug effects* / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D007223:Infant; D007888:Leigh Disease / Q000378:metabolism*; D008297:Male; D017319:Photosensitizing Agents / Q000494:pharmacology*; D012256:Riboflavin / Q000494:pharmacology*
D017319:Photosensitizing Agents; D000255:Adenosine Triphosphate; D012256:Riboflavin