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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 (<jats:italic>Gnpat<\/jats:italic> KO). In\u2010vivo electrocardiographic recordings showed that the duration of the QRS complex was significantly longer in <jats:italic>Gnpat<\/jats:italic> KO mice than in age\u2010 and sex\u2010matched wild\u2010type animals, indicative of reduced cardiac conduction velocity. Oral supplementation of BA for 2\u2009months resulted in normalization of cardiac Pls levels and of the QRS duration in <jats:italic>Gnpat<\/jats:italic> KO mice but not in untreated animals. BA treatment had no effect on the QRS duration in age\u2010matched wild\u2010type mice. These data suggest that Pls deficiency is associated with increased ventricular conduction time which can be rescued by oral BA supplementation.<\/jats:p>","DOI":"10.1002\/jimd.12264","type":"journal-article","created":{"date-parts":[[2020,5,22]],"date-time":"2020-05-22T09:40:33Z","timestamp":1590140433000},"page":"1046-1055","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Oral batyl alcohol supplementation rescues decreased cardiac conduction in ether phospholipid\u2010deficient mice"],"prefix":"10.1002","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1147-4370","authenticated-orcid":false,"given":"Hannes","family":"Todt","sequence":"first","affiliation":[{"name":"Center for Physiology and Pharmacology, Department of Neurophysiology and Neuropharmacology Medical University of Vienna  Vienna 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Pharmacology, Department of Neurophysiology and Neuropharmacology Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Britta","family":"Bruegger","sequence":"additional","affiliation":[{"name":"Heidelberg University Biochemistry Center Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"L\u00fcchtenborg","sequence":"additional","affiliation":[{"name":"Heidelberg University Biochemistry Center Heidelberg University  Heidelberg Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janine","family":"Ebner","sequence":"additional","affiliation":[{"name":"Center for Physiology and Pharmacology, Department of Neurophysiology and Neuropharmacology Medical University of Vienna  Vienna Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karlheinz","family":"Hilber","sequence":"additional","affiliation":[{"name":"Center for 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