Inborn P-oxidation defects

Mitochondrial cytopathies affect mitochondrial respiration, which may secondarily inhibit p-oxidation, as explained above. Mitochondrial cytopathies may therefore be revealed during the administration of drugs that have mitochondrial effects. Thus, the administration of valproate, which inhibits mitochondrial p-oxidation and pyruvate-supported respiration, may reveal a previously latent mitochondrial cytopathy (Chabrol et al. 1994 Lam et al. 1997 Krahenbiihl et al. 2000). For the same reasons, valproate administration can also reveal an inborn p-oxidation defect (Nj0lstad et al. 1997 Kottlors et al. 2001). 

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