Measurement of the Ratio of Lactate to Pyruvate in Skin Fibroblast Cultures

摘要: Publisher Summary A group of inborn errors metabolism exists that result in the condition chronic lactic academia childhood. Nearly all defects, which can be identified, occur mitochondrial proteins, and many demonstrated skin fibroblast cultures established from patients concerned. One approach to diagnose these defects is measure production lactate pyruvate after incubation glucose-containing medium. The total amount ratio between them different cells with dehydrogenase complex or respiratory chain compared unaffected individuals. This chapter focuses on measurement cultures. accumulated, incubating glucose, give an indication absolute rate lactate. It also a whether flux into compromised by defect dehydrogenase. presents principles; procedures, such as sample preparation, determination lactate, pyruvate; results for experiments describing