Summary
Fatty acid (FA) concentrations were studied in lymphocytes isolated from children with syndromic paucity of interlobular bile ducts (PILBD), Alagille syndrome. The aim of this study was to assess whether the specific FA changes previously observed in fibroblast cultures from such patients were also present in other tissues. Lymphocyte FA, obtained both from controls and patients were studied under two experimental conditions, either after separation of the mononuclear cells or after 48 hours of culture. Freshly isolated lymphocytes from patients presented few FA changes compared to the controls. However, when patient lymphocytes were placed in culture medium for 47 hours, FA changes were amplified compared to those observed in controls; the decrease in the sum of saturated andn-6 polyunsaturated FA of total lipids was significant only in patients, and then-3 FA of phospholipids was strikingly increased in patients (p≤0.001), compared to controls. These results are related to those previously observed in fibroblast cultures and suggest that placing cells in culture could reveal a pre-existing cellular abnormality in patients with PILBD.
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Pina, P., Couturier, M. & Lemonnier, F. Fatty acid content in lymphocytes from children with syndromic paucity of interlobular bile ducts, Alagille syndrome. J Inherit Metab Dis 18, 727–736 (1995). https://doi.org/10.1007/BF02436764
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DOI: https://doi.org/10.1007/BF02436764