{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,14]],"date-time":"2026-03-14T00:31:05Z","timestamp":1773448265851,"version":"3.50.1"},"reference-count":25,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2023,5,16]],"date-time":"2023-05-16T00:00:00Z","timestamp":1684195200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000038","name":"Natural Sciences and Engineering Research Council","doi-asserted-by":"publisher","award":["RGPIN-05368-2019"],"award-info":[{"award-number":["RGPIN-05368-2019"]}],"id":[{"id":"10.13039\/501100000038","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000038","name":"Natural Sciences and Engineering Research Council","doi-asserted-by":"publisher","award":["RGPIN-05864-2019"],"award-info":[{"award-number":["RGPIN-05864-2019"]}],"id":[{"id":"10.13039\/501100000038","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Natural Sciences and Engineering Research Council","award":["RGPIN-05368-2019"],"award-info":[{"award-number":["RGPIN-05368-2019"]}]},{"name":"Natural Sciences and Engineering Research Council","award":["RGPIN-05864-2019"],"award-info":[{"award-number":["RGPIN-05864-2019"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biology"],"abstract":"<jats:p>Barth Syndrome (BTHS) is a rare X-linked genetic disease caused by a mutation in the TAFAZZIN gene, which codes for the protein tafazzin involved in cardiolipin remodeling. Approximately 70% of patients with BTHS exhibit severe infections due to neutropenia. However, neutrophils from BTHS patients have been shown to exhibit normal phagocytosis and killing activity. B lymphocytes play a crucial role in the regulation of the immune system and, when activated, secrete cytokines known to attract neutrophils to sites of infection. We examined the expression of chemokine (C-X-C motif) ligand 1 (CXCL1), a known chemotactic for neutrophils, in Epstein\u2013Barr virus transformed control and BTHS B lymphoblasts. Age-matched control and BTHS B lymphoblasts were incubated with Pseudomonas aeruginosa for 24 h and then cell viability, CD27+, CD24+, CD38+, CD138+ and PD1+ surface marker expression and CXCL1 mRNA expression determined. Cell viability was maintained in lymphoblasts incubated in a ratio of 50:1 bacteria:B cells. Surface marker expression was unaltered between control and BTHS B lymphoblasts. In contrast, CXCL1 mRNA expression was reduced approximately 70% (p &lt; 0.05) in untreated BTHS B lymphoblasts compared to control and approximately 90% (p &lt; 0.05) in bacterial treated BTHS B lymphoblasts compared to the control. Thus, na\u00efve and bacterial-activated BTHS B lymphoblasts exhibit reduced mRNA expression of the neutrophil chemoattractant factor CXCL1. We suggest that impaired bacterial activation of B cells in some BTHS patients could influence neutrophil function via impairing neutrophil recruitment to sites of infection and this could potentially contribute to these infections.<\/jats:p>","DOI":"10.3390\/biology12050730","type":"journal-article","created":{"date-parts":[[2023,5,17]],"date-time":"2023-05-17T02:04:05Z","timestamp":1684289045000},"page":"730","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Reduction in mRNA Expression of the Neutrophil Chemoattract Factor CXCL1 in Pseudomonas aeruginosa Treated Barth Syndrome B Lymphoblasts"],"prefix":"10.3390","volume":"12","author":[{"given":"Hana M.","family":"Zegallai","sequence":"first","affiliation":[{"name":"Department of Pharmacology & Therapeutics, Children\u2019s Hospital Research Institute of Manitoba, University of Manitoba 753 McDermot Avenue, Winnipeg, MB R3E0T6, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3016-786X","authenticated-orcid":false,"given":"Kangmin","family":"Duan","sequence":"additional","affiliation":[{"name":"Department of Oral Biology, University of Manitoba, Winnipeg, MB R3E0T6, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grant M.","family":"Hatch","sequence":"additional","affiliation":[{"name":"Department of Pharmacology & Therapeutics, Children\u2019s Hospital Research Institute of Manitoba, University of Manitoba 753 McDermot Avenue, Winnipeg, MB R3E0T6, Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,5,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/0022-510X(83)90209-5","article-title":"An X-linked mitochondrial disease affecting cardiac muscle, skeletal muscle and neutrophil leucocytes","volume":"62","author":"Barth","year":"1983","journal-title":"J. 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