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The condition is characterized by drastic changes in the composition of cardiolipins, a mitochondria\u2010specific class of phospholipids. Studies examining the impact of tafazzin deficiency on the metabolism of other phospholipids have so far generated inhomogeneous and partly conflicting results. Recent studies showed that the cardiolipin composition in cells and different murine tissues is highly dependent on the surrounding lipid environment. In order to study the relevance of different lipid states and tafazzin function for cardiolipin and phospholipid homeostasis we conducted systematic modulation experiments in a CRISPR\/Cas9 knock\u2010out model for BTHS. We found that\u2014irrespective of tafazzin function\u2014the composition of cardiolipins strongly depends on the nutritionally available lipid pool. Tafazzin deficiency causes a consistent shift towards cardiolipin species with more saturated and shorter acyl chains. Interestingly, the typical biochemical BTHS phenotype in phospholipid profiles of HEK 293T\n                    <jats:italic>TAZ<\/jats:italic>\n                    knock\u2010out cells strongly depends on the cellular lipid context. In response to altered nutritional lipid compositions, we measured more pronounced changes on phospholipids that were largely masked under standard cell culturing conditions, therewith giving a possible explanation for the conflicting results reported so far on BTHS lipid phenotypes.\n                  <\/jats:p>","DOI":"10.1002\/jimd.12433","type":"journal-article","created":{"date-parts":[[2021,9,8]],"date-time":"2021-09-08T02:29:50Z","timestamp":1631068190000},"page":"38-50","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["The lipid environment modulates cardiolipin and phospholipid constitution in wild type and tafazzin\u2010deficient cells"],"prefix":"10.1002","volume":"45","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3457-0955","authenticated-orcid":false,"given":"Gregor","family":"Oemer","sequence":"first","affiliation":[{"name":"Institute of Human Genetics, Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2962-125X","authenticated-orcid":false,"given":"Jakob","family":"Koch","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0988-9411","authenticated-orcid":false,"given":"Yvonne","family":"Wohlfarter","sequence":"additional","affiliation":[{"name":"Institute of Human Genetics, Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3260-9139","authenticated-orcid":false,"given":"Katharina","family":"Lackner","sequence":"additional","affiliation":[{"name":"Institute of Biological Chemistry, Medical University of Innsbruck  Innsbruck Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7822-3915","authenticated-orcid":false,"given":"Rita E. 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