Efficacy of two vitamin E formulations in patients with abetalipoproteinemia and chylomicron retention disease

Cuerq, Charlotte; Hénin, Émilie; Restier, Lioara; Blond, Emilie; Drai, Jocelyne; Marçais, Christophe; Filippo, Mathilde Di; Laveille, Christian; Michalski, Marie‐Caroline; Poinsot, Pierre; Caussy, Cyrielle; Sassolas, Agnès; Moulin, Philippe; Reboul, Emmanuelle; Charrière, Sybil; Lévy, Émile; Lachaux, Alain; Peretti, Noël

doi:10.1194/jlr.m085043

Cited by 20 publications

(14 citation statements)

References 39 publications

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“…These results are consistent with the literature reporting that supplementation with tocofersolan had minimal effect on plasma α-tocopherol in adults with normal intestinal absorption, whereas similar doses of TAC resulted in a significant increase in plasma α–tocopherol [ 27 ]. These data are also consistent with our recently published work that showed that TAC was better absorbed than tocofersolan after a single oral dose in healthy volunteers [ 28 ]. One hypothesis to explain the lower absorption of TPGS compared to the TAC could be the steric hindrance of the PEG chain, which would limit (i) its absorption at the enterocyte level, and (ii) its access to an esterase to release tocopherol.…”

Section: Discussionsupporting

confidence: 93%

Comparison of α-Tocopherol, α-Tocopherol Acetate, and α-Tocopheryl Polyethylene Glycol Succinate 1000 Absorption by Caco-2 TC7 Intestinal Cells

et al. 2020

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(1) Background: vitamin E is often supplemented in the form of tocopherol acetate, but it has poor bioavailability and can fail to correct blood tocopherol concentrations in some patients with severe cholestasis. In this context, α-tocopheryl polyethylene glycol succinate 1000 (TPGS) has been of value, but very little is known about the mechanisms of its absorption. The aim of our work was to evaluate the mechanisms of absorption/secretion of TPGS compared to tocopherol acetate (TAC) and α-tocopherol by human enterocyte-like Caco-2 TC7 cells. (2) Methods: two weeks post-confluence Caco-2 cells were incubated with tocopherol- or TAC- or TPGS-rich mixed micelles up to 24 h and, following lipid extraction, TAC and tocopherol amounts were measured by high performance liquid chromatography (HPLC) in apical, cellular, and basolateral compartments. (3) Results: at equivalent concentrations of tocopherol in the apical side, the amounts of tocopherol secreted at the basolateral pole of Caco-2 cells are (i) significantly greater when the tocopherol is in the free form in the micelles; (ii) intermediate when it is in the TAC form in the micelles (p < 0.001); and (iii) significantly lower with the TPGS form (p < 0.0001). Interestingly, our results show, for the first time, that Caco-2 cells secrete one or more esterified forms of the vitamin contained in TPGS at the basolateral side.

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Section: Discussionsupporting

confidence: 93%

Comparison of α-Tocopherol, α-Tocopherol Acetate, and α-Tocopheryl Polyethylene Glycol Succinate 1000 Absorption by Caco-2 TC7 Intestinal Cells

et al. 2020

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“…In addition, due to the single-center design, the generalization of the gut-microbiome signature in population from other geographical locations is unknown 41 . Finally, the association does not suggest causality, and additional studies are needed to assess whether these microbial species impact gut permeability and/or induce NAFLD progression through cross-talk between serum metabolites and the liver 7,11,42,43 . However, the strengths of the study include a prospective study design, detailed phenotyping of participants using the most accurate non-invasive imaging modalities available, and assessment of accuracy using AUROC in both a derivation and validation cohort.…”

Section: Discussionmentioning

confidence: 99%

A gut microbiome signature for cirrhosis due to nonalcoholic fatty liver disease

Caussy¹,

Tripathi²,

Humphrey³

et al. 2019

Nat Commun

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The presence of cirrhosis in nonalcoholic-fatty-liver-disease (NAFLD) is the most important predictor of liver-related mortality. Limited data exist concerning the diagnostic accuracy of gut-microbiome-derived signatures for detecting NAFLD-cirrhosis. Here we report 16S gut-microbiome compositions of 203 uniquely well-characterized participants from a prospective twin and family cohort, including 98 probands encompassing the entire spectrum of NAFLD and 105 of their first-degree relatives, assessed by advanced magnetic-resonance-imaging. We show strong familial correlation of gut-microbiome profiles, driven by shared housing. We report a panel of 30 features, including 27 bacterial features with discriminatory ability to detect NAFLD-cirrhosis using a Random Forest classifier model. In a derivation cohort of probands, the model has a robust diagnostic accuracy (AUROC of 0.92) for detecting NAFLD-cirrhosis, confirmed in a validation cohort of relatives of proband with NAFLD-cirrhosis (AUROC of 0.87). This study provides evidence for a fecal-microbiome-derived signature to detect NAFLD-cirrhosis.

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“…More surprisingly, acanthocytes (or thorny red blood cells, RBCs) are usually observed on blood smears from patients with abetalipoproteinemia and familial homozygous hypobetalipoproteinemia and to a lesser extent in the heterozygous form ( Whitfield et al, 2004 ; Cuerq et al, 2018 ). Acanthocytes are RBCs with few membrane projections varying in length and presenting a non-uniform distribution along the RBC surface, in contrast to echinocytes which exhibit more regular membrane projections that are evenly distributed on the RBC membrane.…”

Section: Introductionmentioning

confidence: 99%

Impaired Cytoskeletal and Membrane Biophysical Properties of Acanthocytes in Hypobetalipoproteinemia – A Case Study

et al. 2021

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Familial hypobetalipoproteinemia is a metabolic disorder mainly caused by mutations in the apolipoprotein B gene. In its homozygous form it can lead without treatment to severe ophthalmological and neurological manifestations. In contrast, the heterozygous form is generally asymptomatic but associated with a low risk of cardiovascular disease. Acanthocytes or thorny red blood cells (RBCs) are described for both forms of the disease. However, those morphological changes are poorly characterized and their potential consequences for RBC functionality are not understood. Thus, in the present study, we asked whether, to what extent and how acanthocytes from a patient with heterozygous familial hypobetalipoproteinemia could exhibit altered RBC functionality. Acanthocytes represented 50% of the total RBC population and contained mitoTracker-positive surface patches, indicating the presence of mitochondrial fragments. While RBC osmotic fragility, calcium content and ATP homeostasis were preserved, a slight decrease of RBC deformability combined with an increase of intracellular free reactive oxygen species were observed. The spectrin cytoskeleton was altered, showing a lower density and an enrichment in patches. At the membrane level, no obvious modification of the RBC membrane fatty acids nor of the cholesterol content were detected but the ceramide species were all increased. Membrane stiffness and curvature were also increased whereas transversal asymmetry was preserved. In contrast, lateral asymmetry was highly impaired showing: (i) increased abundance and decreased functionality of sphingomyelin-enriched domains; (ii) cholesterol enrichment in spicules; and (iii) ceramide enrichment in patches. We propose that oxidative stress induces cytoskeletal alterations, leading to increased membrane stiffness and curvature and impaired lipid lateral distribution in domains and spicules. In addition, ceramide- and spectrin-enriched patches could result from a RBC maturation defect. Altogether, the data indicate that acanthocytes are associated with cytoskeletal and membrane lipid lateral asymmetry alterations, while deformability is only mildly impaired. In addition, familial hypobetalipoproteinemia might also affect RBC precursors leading to disturbed RBC maturation. This study paves the way for the potential use of membrane biophysics and lipid vital imaging as new methods for diagnosis of RBC disorders.

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Efficacy of two vitamin E formulations in patients with abetalipoproteinemia and chylomicron retention disease

Cited by 20 publications

References 39 publications

Comparison of α-Tocopherol, α-Tocopherol Acetate, and α-Tocopheryl Polyethylene Glycol Succinate 1000 Absorption by Caco-2 TC7 Intestinal Cells

Comparison of α-Tocopherol, α-Tocopherol Acetate, and α-Tocopheryl Polyethylene Glycol Succinate 1000 Absorption by Caco-2 TC7 Intestinal Cells

A gut microbiome signature for cirrhosis due to nonalcoholic fatty liver disease

Impaired Cytoskeletal and Membrane Biophysical Properties of Acanthocytes in Hypobetalipoproteinemia – A Case Study

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