Abstract
Liver triglyceride accumulation is common in cystic fibrosis (CF), results in oxidative stress and is a characteristic feature of choline deficiency due to lack of choline for phosphatidylcholine (PC) synthesis via the CDP-choline. PC is formed from phosphatidylethanolamine using methyl groups from methionine via S-adenosylmethionine (SAM) with generation of S-adenosylhomocysteine (SAH). Previously we showed increased PC excretion, and increased plasma homocysteine and SAH, and decreased SAM/SAH in children with CF. In these studies we determined the relation between choline and methionine-homocysteine in children with CF supplemented with lecithin, 2g/d, n=13, choline, 2g/d n=10 or betaine 3g/d n=10 for 14 days. Plasma thiols and phospholipids were determined before and after supplementation, and in children without CF, n=15. Children with CF had significantly higher plasma homocysteine and SAH and lower methionine and SAM/ SAH and GSH/GSSG ratios than children without CF, P<0.05. Plasma methionine, SAM/SAH and GSH/GSSG ratios were significantly increased by supplementation with lecithin, choline and betaine, n=35. All the supplements increased the SAM/SAH, supplementation with choline increased GSH and GSH/GSSG ratio. We show that dietary supplementation aimed at increasing choline status improves altered thiol metabolism and may reduce oxidant stress in children with cystic fibrosis.
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