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While in PCD the amount of carnitine is insufficient (which impairs energy production from fatty acids), CPT1 and CPT2 deficiency affects the binding of fatty acids to carnitine and their use by the mitochondria as an energy source, as described above

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[DOI] [PubMed] [Google Scholar] Murakami K, Sugimoto T, Woo M, Nishida N, Muro H

Pharmakokinetische Daten Bioverfgbarkeit: 90100% Zeit zwischen Einnahme und maximaler Serumkonzentration (t max ): 18 Stunden Zeit bis zum Erreichen einer Steady-State-Konzentration: 2 Tage Verteilungsvolumen: 0,090,17 l/kg Proteinbindung: 7093% Eliminationshalbwertszeit: 717 Stunden Metabolismus Der Groteil der Valproinsure wird in der Leber abgebaut