{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T10:27:04Z","timestamp":1778754424711,"version":"3.51.4"},"reference-count":34,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["ATVB"],"published-print":{"date-parts":[[2024,8]]},"abstract":"<jats:sec>\n            <jats:title>BACKGROUND:<\/jats:title>\n            <jats:p>\n              Pathogenic variants in\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -encoding PLIN1 (perilipin-1) are responsible for an autosomal dominant form of familial partial lipodystrophy (FPL) associated with severe insulin resistance, hepatic steatosis, and important hypertriglyceridemia. This study aims to decipher the mechanisms of hypertriglyceridemia associated with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>METHODS:<\/jats:title>\n            <jats:p>\n              We performed an in vivo lipoprotein kinetic study in 6 affected patients compared with 13 healthy controls and 8 patients with type 2 diabetes. Glucose and lipid parameters, including plasma LPL (lipoprotein lipase) mass, were measured.\n              <jats:italic toggle=\"yes\">LPL<\/jats:italic>\n              mRNA and protein expression were evaluated in abdominal subcutaneous adipose tissue from patients with 5\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -mutated FPL and 3 controls.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>RESULTS:<\/jats:title>\n            <jats:p>\n              Patients with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -mutated FPL presented with decreased fat mass, insulin resistance, and diabetes (glycated hemoglobin A1c, 6.68\u00b10.70% versus 7.48\u00b11.63% in patients with type 2 diabetes; mean\u00b1SD;\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.27). Their plasma triglycerides were higher (5.96\u00b13.08 mmol\/L) than in controls (0.76\u00b10.27 mmol\/L;\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              &lt;0.0001) and patients with type 2 diabetes (2.94\u00b11.46 mmol\/L,\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.006). Compared with controls, patients with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL had a significant reduction of the indirect fractional catabolic rate of VLDL (very-low-density lipoprotein)-apoB100 toward IDL (intermediate-density lipoprotein)\/LDL (low-density lipoprotein; 1.79\u00b11.38 versus 5.34\u00b12.45 pool\/d;\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.003) and the indirect fractional catabolic rate of IDL-apoB100 toward LDL (2.14\u00b11.44 versus 7.51\u00b14.07 pool\/d;\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.005). VLDL-apoB100 production was not different between patients with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL and controls. Compared with patients with type 2 diabetes, patients with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL also showed a significant reduction of the catabolism of both VLDL-apoB100 (\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.031) and IDL-apoB100 (\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              =0.031). Plasma LPL mass was significantly lower in patients with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL than in controls (21.03\u00b110.08 versus 55.76\u00b113.10 ng\/mL;\n              <jats:italic toggle=\"yes\">P<\/jats:italic>\n              &lt;0.0001), although the LPL protein expression in adipose tissue was similar. VLDL-apoB100 and IDL-apoB100 indirect fractional catabolic rates were negatively correlated with plasma triglycerides and positively correlated with LPL mass.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>CONCLUSIONS:<\/jats:title>\n            <jats:p>\n              We show that hypertriglyceridemia associated with\n              <jats:italic toggle=\"yes\">PLIN1<\/jats:italic>\n              -related FPL results from a marked decrease in the catabolism of triglyceride-rich lipoproteins (VLDL and IDL). This could be due to a pronounced reduction in LPL availability, related to the decreased adipose tissue mass.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/atvbaha.124.320774","type":"journal-article","created":{"date-parts":[[2024,6,20]],"date-time":"2024-06-20T09:00:32Z","timestamp":1718874032000},"page":"1873-1883","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":9,"title":["Hypertriglyceridemia Results From an Impaired Catabolism of Triglyceride-Rich Lipoproteins in\n            <i>PLIN1<\/i>\n            -Related Lipodystrophy"],"prefix":"10.1161","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8957-629X","authenticated-orcid":true,"given":"Bruno","family":"Verg\u00e8s","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Diabetology and Metabolic Diseases (B.V., A.R.), University Hospital, Dijon, France."},{"name":"University of Burgundy, INSERM (Institut national de la sant\u00e9 et de la recherche m\u00e9dicale) CTM (Centre de recherche Translationnelle en M\u00e9decine mol\u00e9culaire) UMR1231, Dijon, France (B.V., L.D., A.R.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marie-Christine","family":"Vantyghem","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology, and Metabolism, University of Lille, CHU (Centre Hospitalier Universitaire) Lille, France (M.C.V.)."},{"name":"Universit\u00e9 Lille, U1190 Translational Research for Diabetes, INSERM, Institut Pasteur de Lille, France (M.C.V.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6267-8058","authenticated-orcid":true,"given":"Yves","family":"Reznik","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, University Hospital, Caen, France (Y.R.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laurence","family":"Duvillard","sequence":"additional","affiliation":[{"name":"Department of Biochemistry (L.D.), University Hospital, Dijon, France."},{"name":"University of Burgundy, INSERM (Institut national de la sant\u00e9 et de la recherche m\u00e9dicale) CTM (Centre de recherche Translationnelle en M\u00e9decine mol\u00e9culaire) UMR1231, Dijon, France (B.V., L.D., A.R.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0727-2052","authenticated-orcid":true,"given":"Alexia","family":"Rouland","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Metabolic Diseases (B.V., A.R.), University Hospital, Dijon, France."},{"name":"University of Burgundy, INSERM (Institut national de la sant\u00e9 et de la recherche m\u00e9dicale) CTM (Centre de recherche Translationnelle en M\u00e9decine mol\u00e9culaire) UMR1231, Dijon, France (B.V., L.D., A.R.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8396-2843","authenticated-orcid":true,"given":"Emilie","family":"Capel","sequence":"additional","affiliation":[{"name":"Inserm U938, Saint-Antoine Research Centre, Institute of Cardiometabolism and Nutrition, Sorbonne University, Paris, France (E.C., C.V.)."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8181-4721","authenticated-orcid":true,"given":"Corinne","family":"Vigouroux","sequence":"additional","affiliation":[{"name":"Inserm U938, Saint-Antoine Research Centre, Institute of Cardiometabolism and Nutrition, Sorbonne University, Paris, France (E.C., C.V.)."},{"name":"Department of Molecular Biology and Genetics (C.V.), Assistance Publique-H\u00f4pitaux de Paris, Saint-Antoine University Hospital, France."},{"name":"Department of Endocrinology, Diabetology and Reproductive Endocrinology, National Reference Center for Rare Diseases of Insulin Secretion and Insulin Sensitivity (C.V.), Assistance Publique-H\u00f4pitaux de Paris, Saint-Antoine University Hospital, 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