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Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice

Plasmalogens (Pls) are a class of membrane phospholipids which serve a number of essential biological functions. Deficiency of Pls is associated with common disorders such as Alzheimer's disease or ischemic heart disease. A complete lack of Pls due to genetically determined defective biosynthes...

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Autores principales: Todt, Hannes, Dorninger, Fabian, Rothauer, Peter J., Fischer, Claus M., Schranz, Michael, Bruegger, Britta, Lüchtenborg, Christian, Ebner, Janine, Hilber, Karlheinz, Koenig, Xaver, Erdem, Fatma A., Gawali, Vaibhavkumar S., Berger, Johannes
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Inc. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7540404/
https://www.ncbi.nlm.nih.gov/pubmed/32441337
http://dx.doi.org/10.1002/jimd.12264
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author Todt, Hannes
Dorninger, Fabian
Rothauer, Peter J.
Fischer, Claus M.
Schranz, Michael
Bruegger, Britta
Lüchtenborg, Christian
Ebner, Janine
Hilber, Karlheinz
Koenig, Xaver
Erdem, Fatma A.
Gawali, Vaibhavkumar S.
Berger, Johannes
author_facet Todt, Hannes
Dorninger, Fabian
Rothauer, Peter J.
Fischer, Claus M.
Schranz, Michael
Bruegger, Britta
Lüchtenborg, Christian
Ebner, Janine
Hilber, Karlheinz
Koenig, Xaver
Erdem, Fatma A.
Gawali, Vaibhavkumar S.
Berger, Johannes
author_sort Todt, Hannes
collection PubMed
description Plasmalogens (Pls) are a class of membrane phospholipids which serve a number of essential biological functions. Deficiency of Pls is associated with common disorders such as Alzheimer's disease or ischemic heart disease. A complete lack of Pls due to genetically determined defective biosynthesis gives rise to rhizomelic chondrodysplasia punctata (RCDP), characterized by a number of severe disabling pathologic features and death in early childhood. Frequent cardiac manifestations of RCDP include septal defects, mitral valve prolapse, and patent ductus arteriosus. In a mouse model of RCDP, reduced nerve conduction velocity was partially rescued by dietary oral supplementation of the Pls precursor batyl alcohol (BA). Here, we examine the impact of Pls deficiency on cardiac impulse conduction in a similar mouse model (Gnpat KO). In‐vivo electrocardiographic recordings showed that the duration of the QRS complex was significantly longer in Gnpat KO mice than in age‐ and sex‐matched wild‐type animals, indicative of reduced cardiac conduction velocity. Oral supplementation of BA for 2 months resulted in normalization of cardiac Pls levels and of the QRS duration in Gnpat KO mice but not in untreated animals. BA treatment had no effect on the QRS duration in age‐matched wild‐type mice. These data suggest that Pls deficiency is associated with increased ventricular conduction time which can be rescued by oral BA supplementation.
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spelling pubmed-75404042020-10-09 Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice Todt, Hannes Dorninger, Fabian Rothauer, Peter J. Fischer, Claus M. Schranz, Michael Bruegger, Britta Lüchtenborg, Christian Ebner, Janine Hilber, Karlheinz Koenig, Xaver Erdem, Fatma A. Gawali, Vaibhavkumar S. Berger, Johannes J Inherit Metab Dis Original Articles Plasmalogens (Pls) are a class of membrane phospholipids which serve a number of essential biological functions. Deficiency of Pls is associated with common disorders such as Alzheimer's disease or ischemic heart disease. A complete lack of Pls due to genetically determined defective biosynthesis gives rise to rhizomelic chondrodysplasia punctata (RCDP), characterized by a number of severe disabling pathologic features and death in early childhood. Frequent cardiac manifestations of RCDP include septal defects, mitral valve prolapse, and patent ductus arteriosus. In a mouse model of RCDP, reduced nerve conduction velocity was partially rescued by dietary oral supplementation of the Pls precursor batyl alcohol (BA). Here, we examine the impact of Pls deficiency on cardiac impulse conduction in a similar mouse model (Gnpat KO). In‐vivo electrocardiographic recordings showed that the duration of the QRS complex was significantly longer in Gnpat KO mice than in age‐ and sex‐matched wild‐type animals, indicative of reduced cardiac conduction velocity. Oral supplementation of BA for 2 months resulted in normalization of cardiac Pls levels and of the QRS duration in Gnpat KO mice but not in untreated animals. BA treatment had no effect on the QRS duration in age‐matched wild‐type mice. These data suggest that Pls deficiency is associated with increased ventricular conduction time which can be rescued by oral BA supplementation. John Wiley & Sons, Inc. 2020-06-05 2020-09 /pmc/articles/PMC7540404/ /pubmed/32441337 http://dx.doi.org/10.1002/jimd.12264 Text en © 2020 The Authors. Journal of Inherited Metabolic Disease published by John Wiley & Sons Ltd on behalf of SSIEM This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Articles
Todt, Hannes
Dorninger, Fabian
Rothauer, Peter J.
Fischer, Claus M.
Schranz, Michael
Bruegger, Britta
Lüchtenborg, Christian
Ebner, Janine
Hilber, Karlheinz
Koenig, Xaver
Erdem, Fatma A.
Gawali, Vaibhavkumar S.
Berger, Johannes
Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title_full Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title_fullStr Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title_full_unstemmed Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title_short Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
title_sort oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
topic Original Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7540404/
https://www.ncbi.nlm.nih.gov/pubmed/32441337
http://dx.doi.org/10.1002/jimd.12264
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