Krauß Stephanie, Michaelis Lea, Vetter Walter
Institute of Food Chemistry, University of Hohenheim, Stuttgart, Germany.
PLoS One. 2017 Nov 13;12(11):e0188035. doi: 10.1371/journal.pone.0188035. eCollection 2017.
Patients suffering from Refsum's disease show mutations in the enzyme necessary for the degradation of phytanic acid. Accumulation of this tetramethyl-branched fatty acid in inner organs leads to severe neurological and cardiac dysfunctions which can even result in death. Thus, patients with Refsum's disease have to follow a specific diet resigning foods with high levels of phytanic acid and trans-phytol like products from ruminant animals with a tolerable daily intake (TDI) of ≤ 10 mg/d. We recently reported the occurrence of phytyl fatty acid esters (PFAE, trans-phytol esterified with a fatty acid) in bell pepper with trans-phytol amounts of up to 5.4 mg/100 g fresh weight (FW). In this study we carried out in vitro-digestion experiments of PFAE with artificial digestion fluids. Our results demonstrate that PFAE actually are a source for bioavailable trans-phytol and thus add to the TDI. Eating only one portion of bell pepper (∼150 g) could therefore lead to exploitation of the TDI of up to 81%. Analysis of additional vegetable matrices showed that also rocket salad with up to 4.2 mg/100 g FW trans-phytol bound in PFAE represents a risk-relevant food for patients with Refsum's disease and should therefore be taken into account.
患有雷夫叙姆病的患者体内，植烷酸降解所需的酶存在突变。这种四甲基支链脂肪酸在体内器官中的积累会导致严重的神经和心脏功能障碍，甚至可能导致死亡。因此，雷夫叙姆病患者必须遵循特定饮食，避免食用富含植烷酸和反式植醇的食物，如反刍动物产品，每日可耐受摄入量（TDI）≤10毫克/天。我们最近报道，甜椒中存在植基脂肪酸酯（PFAE，反式植醇与脂肪酸酯化产物），反式植醇含量高达5.4毫克/100克鲜重（FW）。在本研究中，我们用人工消化液对PFAE进行了体外消化实验。我们的结果表明，PFAE实际上是生物可利用反式植醇的一个来源，因此会增加TDI。因此，仅食用一份甜椒（约150克）就可能导致TDI超标81%。对其他蔬菜基质的分析表明，火箭生菜中PFAE结合的反式植醇含量高达4.2毫克/100克FW，对雷夫叙姆病患者来说也是一种与风险相关的食物，因此应予以考虑。
