Enzymatic preparation and facile purification of medium-chain, and medium- and long-chain fatty acid diacylglycerols

LWT ◽  
2018 ◽  
Vol 92 ◽  
pp. 227-233 ◽  
Author(s):  
Guanghui Li ◽  
Jiazi Chen ◽  
Xiang Ma ◽  
Zhen Zhang ◽  
Ning Liu ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Chain Fatty Acid ◽  
Enzymatic Preparation ◽  
Long Chain Fatty Acid ◽  
Medium Chain ◽  
Long Chain

scholarly journals Lipidomic and Proteomic Alterations Induced by Even and Odd Medium-Chain Fatty Acids on Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders

2021 ◽  
Vol 22 (19) ◽  
pp. 10556
Author(s):  
Khaled I. Alatibi ◽  
Stefan Tholen ◽  
Zeinab Wehbe ◽  
Judith Hagenbuchner ◽  
Daniela Karall ◽  
...  
Keyword(s):  
Fatty Acids ◽  
Fatty Acid ◽  
Fatty Acid Oxidation ◽  
Chain Fatty Acid ◽  
Acid Oxidation ◽  
Long Chain Fatty Acid ◽  
Fatty Acid Oxidation Disorders ◽  
Medium Chain ◽  
Medium Chain Fatty Acids ◽  
Long Chain

Medium-chain fatty acids (mc-FAs) are currently applied in the treatment of long-chain fatty acid oxidation disorders (lc-FAOD) characterized by impaired β-oxidation. Here, we performed lipidomic and proteomic analysis in fibroblasts from patients with very long-chain acyl-CoA dehydrogenase (VLCADD) and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHADD) deficiencies after incubation with heptanoate (C7) and octanoate (C8). Defects of β-oxidation induced striking proteomic alterations, whereas the effect of treatment with mc-FAs was minor. However, mc-FAs induced a remodeling of complex lipids. Especially C7 appeared to act protectively by restoring sphingolipid biosynthesis flux and improving the observed dysregulation of protein homeostasis in LCHADD under control conditions.

The safety of Lipistart, a medium-chain triglyceride based formula, in the dietary treatment of long-chain fatty acid disorders: a phase I study

2018 ◽  
Vol 31 (3) ◽  
pp. 297-304
Author(s):  
Anita MacDonald ◽  
Rachel Webster ◽  
Matthew Whitlock ◽  
Adam Gerrard ◽  
Anne Daly ◽  
...  
Keyword(s):  
Fatty Acid ◽  
Linoleic Acid ◽  
Phase I ◽  
Dehydrogenase Deficiency ◽  
Medium Chain Triglyceride ◽  
Chain Fatty Acid ◽  
Infants And Children ◽  
Long Chain Fatty Acid ◽  
Medium Chain ◽  
Long Chain

Abstract Background: Children with long-chain fatty acid β-oxidation disorders (LCFAOD) presenting with clinical symptoms are treated with a specialist infant formula, with medium chain triglyceride (MCT) mainly replacing long chain triglyceride (LCT). It is essential that the safety and efficacy of any new specialist formula designed for LCFAOD be tested in infants and children. Methods: In an open-label, 21-day, phase I trial, we studied the safety of a new MCT-based formula (feed 1) in six well-controlled children (three male), aged 7–13 years (median 9 years) with LCFAOD (very long chain acyl CoA dehydrogenase deficiency [VLCADD], n=2; long chain 3-hydroxyacyl CoA dehydrogenase deficiency [LCHADD], n=2; carnitine acyl carnitine translocase deficiency [CACTD], n=2). Feed 1 (Lipistart; Vitaflo) contained 30% energy from MCT, 7.5% LCT and 3% linoleic acid and it was compared with a conventional MCT feed (Monogen; Nutricia) (feed 2) containing 17% energy from MCT, 3% LCT and 1.1% linoleic acid. Subjects consumed feed 2 for 7 days then feed 1 for 7 days and finally resumed feed 2 for 7 days. Vital signs, blood biochemistry, ECG, weight, height, food/feed intake and symptoms were monitored. Results: Five subjects completed the study. Their median daily volume of both feeds was 720 mL (range 500–1900 mL/day). Feed 1 was associated with minimal changes in tolerance, free fatty acids (FFA), acylcarnitines, 3-hydroxybutyrate (3-HB), creatine kinase (CK), blood glucose, liver enzymes and no change in an electrocardiogram (ECG). No child complained of muscle pain or symptoms associated with LCFAOD on either feed. Conclusions: This is the first safety trial reported of an MCT formula specifically designed for infants and children with LCFAOD. In this short-term study, it appeared safe and well tolerated in this challenging group.

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