Phosphatidylethanolamine homeostasis under conditions of impaired CDP-ethanolamine pathway or phosphatidylserine decarboxylation

Germain, Michaela St; Iraji, Roya; Bakovic, Marica

doi:10.3389/fnut.2022.1094273

Cited by 9 publications

(2 citation statements)

References 215 publications

(355 reference statements)

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“…Furthermore, the plasma from MCADD patients exhibited decreased levels of three species of phosphatidylethanolamine (PE), which is recognized as the second most abundant class of phospholipids in blood plasma. It is well‐known that PE can be converted into PC lipids through the Kennedy pathway by the action of PE N‐methyltransferase (PEMT) 109,110 . The decreased levels of PE species in MCADD patients may be correlated with the increase in PC levels observed in this study.…”

Section: Discussionsupporting

confidence: 63%

“…It is well-known that PE can be converted into PC lipids through the Kennedy pathway by the action of PE N-methyltransferase (PEMT). 109,110 The decreased levels of PE species in MCADD patients may be correlated with the increase in PC levels observed in this study. In the future, it would be important to evaluate the activity of the PEMT enzyme in MCADD patients.…”

Section: Lipid Speciessupporting

confidence: 54%

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Plasma lipidomics analysis reveals altered profile of triglycerides and phospholipids in children with Medium‐Chain Acyl‐CoA dehydrogenase deficiency

Guerra,

Ferreira,

Maurício

et al. 2024

J of Inher Metab Disea

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Medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD) is the most prevalent mitochondrial fatty acid β‐oxidation disorder. In this study, we assessed the variability of the lipid profile in MCADD by analysing plasma samples obtained from 25 children with metabolically controlled MCADD (following a normal diet with frequent feeding and under l‐carnitine supplementation) and 21 paediatric control subjects (CT). Gas chromatography–mass spectrometry was employed for the analysis of esterified fatty acids, while high‐resolution C18‐liquid chromatography‐mass spectrometry was used to analyse lipid species. We identified a total of 251 lipid species belonging to 15 distinct lipid classes. Principal component analysis revealed a clear distinction between the MCADD and CT groups. Univariate analysis demonstrated that 126 lipid species exhibited significant differences between the two groups. The lipid species that displayed the most pronounced variations included triacylglycerols and phosphatidylcholines containing saturated and monounsaturated fatty acids, specifically C14:0 and C16:0, which were found to be more abundant in MCADD. The observed changes in the plasma lipidome of children with non‐decompensated MCADD suggest an underlying alteration in lipid metabolism. Therefore, longitudinal monitoring and further in‐depth investigations are warranted to better understand whether such alterations are specific to MCADD children and their potential long‐term impacts.

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Section: Discussionsupporting

confidence: 63%

Section: Lipid Speciessupporting

confidence: 54%

Plasma lipidomics analysis reveals altered profile of triglycerides and phospholipids in children with Medium‐Chain Acyl‐CoA dehydrogenase deficiency

Guerra,

Ferreira,

Maurício

et al. 2024

J of Inher Metab Disea

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Mitochondrial membrane synthesis, remodelling and cellular trafficking

Messina,

Vaz,

Rahman

2024

J of Inher Metab Disea

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Mitochondria are dynamic cellular organelles with complex roles in metabolism and signalling. Primary mitochondrial disorders are a group of approximately 400 monogenic disorders arising from pathogenic genetic variants impacting mitochondrial structure, ultrastructure and/or function. Amongst these disorders, defects of complex lipid biosynthesis, especially of the unique mitochondrial membrane lipid cardiolipin, and membrane biology are an emerging group characterised by clinical heterogeneity, but with recurrent features including cardiomyopathy, encephalopathy, neurodegeneration, neuropathy and 3‐methylglutaconic aciduria. This review discusses lipid synthesis in the mitochondrial membrane, the mitochondrial contact site and cristae organising system (MICOS), mitochondrial dynamics and trafficking, and the disorders associated with defects of each of these processes. We highlight overlapping functions of proteins involved in lipid biosynthesis and protein import into the mitochondria, pointing to an overarching coordination and synchronisation of mitochondrial functions. This review also focuses on membrane interactions between mitochondria and other organelles, namely the endoplasmic reticulum, peroxisomes, lysosomes and lipid droplets. We signpost disorders of these membrane interactions that may explain the observation of secondary mitochondrial dysfunction in heterogeneous pathological processes. Disruption of these organellar interactions ultimately impairs cellular homeostasis and organismal health, highlighting the central role of mitochondria in human health and disease.

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Characterising the urinary acylcarnitine and amino acid profiles of HIV/TB co-infection, using LC–MS metabolomics

Pretorius,

Luies

2024

Metabolomics

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Introduction The human immunodeficiency virus (HIV) and tuberculosis (TB) co-infection presents significant challenges due to the complex interplay between these diseases, leading to exacerbated metabolic disturbances. Understanding these metabolic profiles is crucial for improving diagnostic and therapeutic approaches. Objective This study aimed to characterise the urinary acylcarnitine and amino acid profiles, including 5-hydroxyindoleacetic acid (5-HIAA), in patients co-infected with HIV and TB using targeted liquid chromatography mass spectrometry (LC–MS) metabolomics. Methods Urine samples, categorised into HIV, TB, HIV/TB co-infected, and healthy controls, were analysed using HPLC–MS/MS. Statistical analyses included one-way ANOVA and a Kruskal-Wallis test to determine significant differences in the acylcarnitine and amino acid profiles between groups. Results The study revealed significant metabolic alterations, especially in TB and co-infected groups. Elevated levels of medium-chain acylcarnitines indicated increased fatty acid oxidation, commonly associated with cachexia in TB. Altered amino acid profiles suggested disruptions in protein and glucose metabolism, indicating a shift towards diabetes-like metabolic states. Notably, TB was identified as a primary driver of these changes, affecting protein turnover, and impacting energy metabolism in co-infected patients. Conclusion The metabolic profiling of HIV/TB co-infection highlights the profound impact of TB on metabolic pathways, which may exacerbate the clinical complexities of co-infection. Understanding these metabolic disruptions can guide the development of targeted treatments and improve management strategies, ultimately enhancing the clinical outcomes for these patients. Further research is required to validate these findings and explore their implications in larger, diverse populations.

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Phosphatidylethanolamine homeostasis under conditions of impaired CDP-ethanolamine pathway or phosphatidylserine decarboxylation

Cited by 9 publications

References 215 publications

Plasma lipidomics analysis reveals altered profile of triglycerides and phospholipids in children with Medium‐Chain Acyl‐CoA dehydrogenase deficiency

Plasma lipidomics analysis reveals altered profile of triglycerides and phospholipids in children with Medium‐Chain Acyl‐CoA dehydrogenase deficiency

Mitochondrial membrane synthesis, remodelling and cellular trafficking

Characterising the urinary acylcarnitine and amino acid profiles of HIV/TB co-infection, using LC–MS metabolomics

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