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Ibuprofen is a competitive inhibitor of prostaglandin (PG) synthesis through inhibition of cyclooxygenase (COX), which results in decreased production of prostaglandin I2 and prostaglandin E2

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Interestingly, it has been found that this syndrome can also be caused by a mutation in the mitochondrial ribosomal protein MRPS22 with deficiencies of OXPHOS complexes I, III, and IV in fibroblast mitochondria [392]