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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Veröffentlicht in:Journal of inherited metabolic disease 2020-09, Vol.43 (5), p.1046-1055
Hauptverfasser: 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
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container_end_page 1055
container_issue 5
container_start_page 1046
container_title Journal of inherited metabolic disease
container_volume 43
creator 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
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.
doi_str_mv 10.1002/jimd.12264
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subjects Alzheimer's disease
arrhythmia
batyl alcohol
cardiac impulse conduction
Children
Chondrodystrophy
Coronary artery disease
Dietary supplements
electrocardiography
Heart diseases
Ischemia
Mitral valve
Nerve conduction
Neurodegenerative diseases
Original
Phospholipids
Plasmalogens
Rheumatic heart disease
rhizomelic chondrodysplasia punctata
Velocity
Ventricle
title Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid‐deficient mice
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