Premium
Fatty acid content in lymphocytes from children with syndromic paucity of interlobular bile ducts, Alagille syndrome
Author(s) -
Pina P.,
Couturier M.,
Lemonnier F.
Publication year - 1995
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf02436764
Subject(s) - alagille syndrome , lymphocyte , medicine , fibroblast , endocrinology , abnormality , polyunsaturated fatty acid , peripheral blood mononuclear cell , fatty acid , biology , pathology , gastroenterology , immunology , biochemistry , in vitro , cholestasis , psychiatry
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 and n ‐6 polyunsaturated FA of total lipids was significant only in patients, and the n ‐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.