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Differential inhibitory effect of long-chain acyl-CoA esters on succinate and g...

Ventura, FV; Ruiter, J; Ijlst, L; de Almeida, IT; Wanders, RJA

Long-chain fatty acid-oxidation defects are associated with a series of clinical and biochemical abnormalities, including accumulation of long-chain acyl-CoA esters which have been shown to inhibit several enzymes and transport systems that may disturb en


Valproyl-dephosphocoa

Silva, MFB; Ijlst, L; Allers, P; Jakobs, C; Duran, M; de Almeida, IT; Wanders, RJA

The mitochondrial metabolism of valproic acid (VPA) was investigated in vitro to elucidate its beta-oxidation pathway since the characterization of VPA intermediates in the acyl-CoA thioester form, and not just in their free acid form, has not been fully


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