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Although a direct causal relationship between the copper toxicity and the formation of PA or PHA is lacking, studies by high-performance thin-layer chromatography and MALDI-TOF-MS (Matrix-Assisted Laser Desorption/Ionization Time of Flight Mass Spectrometry) have revealed an increased level of PA and PHA in the liver mitochondria of ATP7b −/− mice but not the wild-type control mice [53]