{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,6]],"date-time":"2025-11-06T12:25:43Z","timestamp":1762431943959,"version":"build-2065373602"},"reference-count":29,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2021,1,23]],"date-time":"2021-01-23T00:00:00Z","timestamp":1611360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100000361","name":"Diabetes UK","doi-asserted-by":"publisher","award":["18\/0005884","17\/0005621"],"award-info":[{"award-number":["18\/0005884","17\/0005621"]}],"id":[{"id":"10.13039\/501100000361","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/L002620\/1"],"award-info":[{"award-number":["MR\/L002620\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["PG\/14\/43\/30889"],"award-info":[{"award-number":["PG\/14\/43\/30889"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010661","name":"Horizon 2020","doi-asserted-by":"publisher","award":["2016-726152-TYPHI"],"award-info":[{"award-number":["2016-726152-TYPHI"]}],"id":[{"id":"10.13039\/100010661","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Congenital Generalized Lipodystrophy type 2 (CGL2) is the most severe form of lipodystrophy and is caused by mutations in the BSCL2 gene. Affected patients exhibit a near complete lack of adipose tissue and suffer severe metabolic disease. A recent study identified infection as a major cause of death in CGL2 patients, leading us to examine whether Bscl2 loss could directly affect the innate immune response. We generated a novel mouse model selectively lacking Bscl2 in the myeloid lineage (LysM-B2KO) and also examined the function of bone-marrow-derived macrophages (BMDM) isolated from global Bscl2 knockout (SKO) mice. LysM-B2KO mice failed to develop lipodystrophy and metabolic disease, providing a model to study the direct role of Bscl2 in myeloid lineage cells. Lipopolysaccharide-mediated stimulation of inflammatory cytokines was not impaired in LysM-B2KO mice or in BMDM isolated from either LysM-B2KO or SKO mice. Additionally, intracellular fate and clearance of bacteria in SKO BMDM challenged with Staphylococcus aureus was indistinguishable from that in BMDM isolated from littermate controls. Overall, our findings reveal that selective Bscl2 deficiency in macrophages does not critically impact the innate immune response to infection. Instead, an increased susceptibility to infection in CGL2 patients is likely to result from severe metabolic disease.<\/jats:p>","DOI":"10.3390\/jcm10030441","type":"journal-article","created":{"date-parts":[[2021,1,25]],"date-time":"2021-01-25T02:07:00Z","timestamp":1611540420000},"page":"441","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Bscl2 Deficiency Does Not Directly Impair the Innate Immune Response in a Murine Model of Generalized Lipodystrophy"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6831-4426","authenticated-orcid":false,"given":"Ahlima","family":"Roumane","sequence":"first","affiliation":[{"name":"The Rowett Institute, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"},{"name":"The Aberdeen Cardiovascular and Diabetes Centre, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"},{"name":"Curtin Health Innovation Research Institute, School of Pharmacy and Biomedical Sciences, Curtin University, Perth, WA 6102, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4230-4355","authenticated-orcid":false,"given":"George D.","family":"Mcilroy","sequence":"additional","affiliation":[{"name":"The Rowett Institute, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"},{"name":"The Aberdeen Cardiovascular and Diabetes Centre, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1887-821X","authenticated-orcid":false,"given":"Arda","family":"Balci","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Weiping","family":"Han","sequence":"additional","affiliation":[{"name":"Laboratory of Metabolic Medicine, Institute of Molecular and Cell Biology, Agency for Science, Technology and Research (A*STAR), Singapore 138673, Singapore"},{"name":"Center for Neuro-Metabolism and Regeneration Research, Guangzhou Regenerative Medicine and Health Guangdong Laboratory, Guangzhou 510700, China"},{"name":"School of Laboratory Medicine and Life Sciences, Wenzhou Medical University, Wenzhou 325035, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mirela","family":"Delibegovi\u0107","sequence":"additional","affiliation":[{"name":"The Aberdeen Cardiovascular and Diabetes Centre, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"},{"name":"Institute of Medical Sciences, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2498-4767","authenticated-orcid":false,"given":"Massimiliano","family":"Baldassarre","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0500-6984","authenticated-orcid":false,"given":"Philip","family":"Newsholme","sequence":"additional","affiliation":[{"name":"Curtin Health Innovation Research Institute, School of Pharmacy and Biomedical Sciences, Curtin University, Perth, WA 6102, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5020-4939","authenticated-orcid":false,"given":"Justin J.","family":"Rochford","sequence":"additional","affiliation":[{"name":"The Rowett Institute, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"},{"name":"The Aberdeen Cardiovascular and Diabetes Centre, University of Aberdeen, Foresterhill, Aberdeen AB25 2ZD, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,1,23]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"3313","DOI":"10.1210\/jc.2011-1159","article-title":"Lipodystrophies: Genetic and acquired body fat disorders","volume":"96","author":"Garg","year":"2011","journal-title":"J. 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