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- W2051103651 abstract "Summary Intact cultured fibroblasts from patients with deficiency of long‐chain 3‐hydroxyacyl‐CoA dehydrogenase release 3 H 2 O from [9,10‐ 3 H]myristic acid and [9,10‐ 3 H]palmitic acid more slowly than normal. The ratio of activity (palmitate/myristate) is also low and the expression (rate with palmitate) 2 /(rate with myristate) gives good differentiation between affected and unaffected cells. In some patients who have shown hydroxydicarboxylic aciduria when unwell there is reduced 3 H 2 O production from [9,10‐ 3 H]myristic and [9,10‐ 3 H]palmitic acids by intact cultured fibroblasts but normal 3‐hydroxyacyl‐CoA dehydrogenase activities in disrupted cells. The palmitate/myristate ratio is higher than in long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency. The basic defect in these patients is still unknown but it is suggested that caution be used over the administration of medium‐chain triglyceride." @default.
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- W2051103651 title "Differential diagnosis of hydroxydicarboxylic aciduria based on release of <sup>3</sup> H <sub>2</sub> O from [9,10‐ <sup>3</sup> H]myristic and [9,10‐ <sup>3</sup> H]palmitic acids by intact cultured fibroblasts" @default.
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- W2051103651 doi "https://doi.org/10.1007/bf01800227" @default.
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