The combined data of four different experiments revealed striking, highly significant differences between control fibroblast and four selected LCHADD patient fibroblasts: LCHADD fibroblasts had only 40–60% basal mitochondrial respiration compared to fibroblasts from healthy controls.
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Very long-/ and long Chain-3-Hydroxy Acyl CoA Dehydrogenase Deficiency correlates with deregulation of the mitochondrial fusion/fission machinery.
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