{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T22:07:19Z","timestamp":1791151639319,"version":"4.1.0"},"reference-count":33,"publisher":"National Academy of Sciences","issue":"7","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2009,2,17]]},"abstract":"<jats:p>\n                    Quantitative and qualitative alterations of mitochondrial cardiolipin have been implicated in the pathogenesis of Barth syndrome, an X-linked cardioskeletal myopathy caused by a deficiency in tafazzin, an enzyme in the cardiolipin remodeling pathway. We have generated and previously reported a tafazzin-deficient\n                    <jats:italic>Drosophila<\/jats:italic>\n                    model of Barth syndrome that is characterized by low cardiolipin concentration, abnormal cardiolipin fatty acyl composition, abnormal mitochondria, and poor motor function. Here, we first show that tafazzin deficiency in\n                    <jats:italic>Drosophila<\/jats:italic>\n                    disrupts the final stage of spermatogenesis, spermatid individualization, and causes male sterility. This phenotype can be genetically suppressed by inactivation of the gene encoding a calcium-independent phospholipase A\n                    <jats:sub>2<\/jats:sub>\n                    , iPLA2-VIA, which also prevents cardiolipin depletion\/monolysocardiolipin accumulation, although in wild-type flies inactivation of the iPLA2-VIA does not affect the molecular composition of cardiolipin. Furthermore, we show that treatment of Barth syndrome patients' lymphoblasts in tissue culture with the iPLA\n                    <jats:sub>2<\/jats:sub>\n                    inhibitor, bromoenol lactone, partially restores their cardiolipin homeostasis. Taken together, these findings establish a causal role of cardiolipin deficiency in the pathogenesis of Barth syndrome and identify iPLA2-VIA as an important enzyme in cardiolipin deacylation, and as a potential target for therapeutic intervention.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0811224106","type":"journal-article","created":{"date-parts":[[2009,1,21]],"date-time":"2009-01-21T21:40:22Z","timestamp":1232574022000},"page":"2337-2341","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":135,"title":["Role of calcium-independent phospholipase A\n                    <sub>2<\/sub>\n                    in the pathogenesis of Barth syndrome"],"prefix":"10.1073","volume":"106","author":[{"given":"Ashim","family":"Malhotra","sequence":"first","affiliation":[{"name":"Anesthesiology, New York University Langone Medical Center, New York, NY 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