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One remarkable abnormality, not observed in, for instance, medium\u2010chain acyl\u2010CoA dehydrogenase deficiency, is the moderate to severe lactic acidaemia in long\u2010chain fatty acid \u03b2\u2010oxidation\u2010deficient patients, suggesting that oxidation of pyruvate is also compromised. In order to understand the underlying basis of the lactic acidaemia in these patients, we have studied the formation of L\u2010lactate and pyruvate in cultured skin fibroblasts incubated with D\u2010glucose. All long\u2010chain fatty acid \u03b2\u2010oxidation\u2010deficient cell lines studied were found to show a moderate elevation of lactate when compared with control and medium\u2010chain acyl\u2010CoA dehydrogenase\u2010deficient fibroblasts. Interestingly, differences were found between cells deficient in long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase and very\u2010long\u2010chain acyl\u2010CoA dehydrogenase, suggesting that saturated acyl\u2010CoA esters and their 3\u2010hydroxyacyl\u2010CoA derivatives affect pyruvate metabolism differently.<\/jats:p>","DOI":"10.1023\/a:1005480516801","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T22:25:23Z","timestamp":1040509523000},"page":"645-654","source":"Crossref","is-referenced-by-count":36,"title":["Lactic acidosis in long\u2010chain fatty acid \u03b2\u2010oxidation disorders"],"prefix":"10.1002","volume":"21","author":[{"given":"F. V.","family":"Ventura","sequence":"first","affiliation":[]},{"given":"J. P. 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