Kang E S, Capaci M T, Sawhney B, Greenhaw J
Biochem Med Metab Biol. 1986 Jun;35(3):361-6. doi: 10.1016/0885-4505(86)90094-0.
To determine whether the accumulation of liver triglyceride in Reye's syndrome could be due to a block in beta-oxidation of the fatty acids, the ability of Reye's and control liver homogenates from samples obtained at autopsy to oxidize fatty acids was examined. Total fatty acid oxidation as measured by oxidation of [1-14C]oleoyl CoA, which mostly represents mitochondrial activity, was comparable between the groups. Peroxisomal fatty acid oxidation was, likewise, similar despite the reported increase in the numbers and sizes of these organelles. This disparity could not be explained by an artifactual dilution of product by accumulated endogenous substrate. Inference is made that active peroxisomal beta-oxidation may contribute to the increased short chain fatty CoA content of liver which was reported earlier.
为了确定瑞氏综合征中肝脏甘油三酯的积累是否可能归因于脂肪酸β-氧化的阻断,研究了取自尸检样本的瑞氏综合征和对照肝脏匀浆氧化脂肪酸的能力。通过[1-14C]油酰辅酶A的氧化来测量的总脂肪酸氧化,这主要代表线粒体活性,在两组之间相当。尽管有报道称这些细胞器的数量和大小有所增加,但过氧化物酶体脂肪酸氧化同样相似。这种差异不能用积累的内源性底物对产物的人为稀释来解释。由此推断,活跃的过氧化物酶体β-氧化可能导致了先前报道的肝脏中短链脂肪酰辅酶A含量的增加。