Respiratory chain analysis of skin fibroblasts in mitochondrial disease

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• 作者：Cameron ; Jessie M. ; Levandovskiy ; Valeriy ; MacKay ; Neviana ; Robinson ; Brian H.
• 关键词：Respiratory chain ; Mitochondria ; Leigh syndrome ; L/P ; lactate to pyruvate ratio ; NADH ; nicotinamide dehydrogenase ; CI ; complex I ; CIII ; complex III ; CIV ; complex IV ; CS ; citrate synthase ; PDH ; pyruvate dehydrogenase
• 刊名：Mitochondrion
• 出版年：2004
• 出版时间：September, 2004
• 年：2004
• 卷：4
• 期：5-6
• 页码：387-394
• 全文大小：181 K

文摘

NADH:ubiquinone dehydrogenase (complex I) deficiency can be diagnosed from cultured skin fibroblasts using a number of methods, the most commonly used is a linked assay of rotenone-sensitive complex I+III activity (NADH:cytochrome c reductase). Because of interference from diaphorases, this method requires either the isolation of mitochondria (or at least partial purification). For a suitable mitochondrial preparation from skin fibroblasts, this requires the culturing of 4–20 individual 100mm tissue culture plates, depending on the purity of preparation required.