{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T17:37:29Z","timestamp":1770831449883,"version":"3.50.1"},"reference-count":50,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["ATVB"],"published-print":{"date-parts":[[1998,4]]},"abstract":"<jats:p>\n            <jats:italic>Abstract<\/jats:italic>\n            \u2014We have previously described patients with familial high density lipoprotein (HDL) deficiency (FHD) having a marked reduction in the plasma concentration of HDL cholesterol and apolipoprotein (apo) A-I but lacking clinical manifestations of Tangier disease or evidence of other known causes of HDL deficiency. To determine whether FHD in these individuals was associated with impaired HDL production or increased HDL catabolism, we investigated the kinetics of plasma apoA-I and apoA-II in two related FHD patients (plasma apoA-I, 17 and 37 mg\/dL) and four control subjects (apoA-I, 126\u00b118 mg\/dL, mean\u00b1SD) by using a primed constant infusion of deuterated leucine. Kinetic analysis of plasma apolipoprotein enrichment curves demonstrated that mature plasma apoA-I production rates (PRs) were similar in patients and control subjects (7.9 and 9.1 versus 10.5\u00b11.7 mg \u00b7 kg\n            <jats:sup>\u22121<\/jats:sup>\n            \u00b7 d\n            <jats:sup>\u22121<\/jats:sup>\n            ). Residence times (RTs) of mature apoA-I were, however, significantly less in FHD patients (0.79 and 1.66 days) compared with controls (5.32\u00b11.05 days). Essentially normal levels of plasma proapoA-I (the precursor protein of apoA-I) in FHD patients were associated with normal plasma proapoA-I PRs (7.8 and 10.4 versus 10.9\u00b12.6 mg \u00b7 kg\n            <jats:sup>\u22121<\/jats:sup>\n            \u00b7 d\n            <jats:sup>\u22121<\/jats:sup>\n            ) and proapoA-I RTs (0.18 and 0.15 versus 0.16\u00b10.03 day). The RTs of apoA-II were, however, less in patients (3.17 and 2.92 days) than control subjects (7.24\u00b10.71 days), whereas the PRs of apoA-II were similar (1.8 and 1.9 versus 1.7\u00b10.2 mg \u00b7 kg\n            <jats:sup>\u22121<\/jats:sup>\n            \u00b7 d\n            <jats:sup>\u22121<\/jats:sup>\n            ). Increased plasma catabolism of apoA-II in FHD patients was associated with the presence in plasma of abnormal apoA-II\u2013HDL (without apoA-I). These results demonstrate that FHD in our patients is characterized, like Tangier disease, by hypercatabolism of mature apoA-I and apoA-II, but unlike Tangier disease, by essentially normal plasma catabolism and concentration of proapoA-I.\n          <\/jats:p>","DOI":"10.1161\/01.atv.18.4.655","type":"journal-article","created":{"date-parts":[[2011,6,17]],"date-time":"2011-06-17T19:44:19Z","timestamp":1308339859000},"page":"655-664","source":"Crossref","is-referenced-by-count":54,"title":["Familial HDL Deficiency Characterized by Hypercatabolism of Mature ApoA-I but Not ProApoA-I"],"prefix":"10.1161","volume":"18","author":[{"given":"Rami","family":"Batal","sequence":"first","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michel","family":"Tremblay","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Larbi","family":"Krimbou","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Orval","family":"Mamer","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean","family":"Davignon","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"suffix":"Jr","given":"Jacques","family":"Genest","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey S.","family":"Cohn","sequence":"additional","affiliation":[{"name":"From the Hyperlipidemia and Atherosclerosis Research Group and the Cardiovascular Genetics Laboratory (J.G.), Clinical Research Institute of Montre\u0301al (R.B., M.T., L.K., J.D., J.S.C.); and the McGill University Biomedical Mass Spectrometry Unit (O.M.), Montre\u0301al, Que\u0301bec, Canada."}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199108083250601"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1986.03380200073024"},{"key":"e_1_3_2_3_2","doi-asserted-by":"crossref","first-page":"1017","DOI":"10.1016\/S0022-2275(20)37713-0","volume":"25","year":"1984","journal-title":"J Lipid Res"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(90)90314-N"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.15.11.1987"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.1.1"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(83)91824-7"},{"key":"e_1_3_2_8_2","first-page":"19","volume":"258","year":"1983","journal-title":"J Biol Chem"},{"key":"e_1_3_2_9_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116384"},{"key":"e_1_3_2_10_2","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/S0022-2275(20)42034-6","volume":"32","year":"1991","journal-title":"J Lipid Res"},{"key":"e_1_3_2_11_2","first-page":"22","volume":"258","year":"1983","journal-title":"J Biol Chem"},{"key":"e_1_3_2_12_2","doi-asserted-by":"crossref","first-page":"12201","DOI":"10.1016\/S0021-9258(20)71339-6","volume":"259","year":"1984","journal-title":"J Biol Chem"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0026-0495(80)90109-2"},{"key":"e_1_3_2_14_2","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1016\/S0022-2275(20)38087-1","volume":"23","year":"1982","journal-title":"J Lipid Res"},{"key":"e_1_3_2_15_2","first-page":"306","volume":"14","year":"1994","journal-title":"Arterioscler Thromb Vasc Biol"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.14.5.707"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.ATV.13.12.1728"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1097\/00041433-199606000-00002"},{"key":"e_1_3_2_19_2","doi-asserted-by":"crossref","unstructured":"Assmann G Funke H. HDL metabolism and atherosclerosis. J Cardiovasc Pharmacol . 1990;16(suppl 9):S15\u2013S20.","DOI":"10.1097\/00005344-199006169-00004"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118082"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.15.5.683"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.15.10.1746"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.15.8.1015"},{"key":"e_1_3_2_24_2","first-page":"117","volume":"36","year":"1994","journal-title":"J Lipid Res"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197810262991701"},{"key":"e_1_3_2_26_2","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1016\/S0022-2275(20)42319-3","volume":"31","year":"1990","journal-title":"J Lipid Res"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI115028"},{"key":"e_1_3_2_28_2","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1016\/S0022-2275(20)41439-7","volume":"33","year":"1992","journal-title":"J Lipid Res"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1016\/0140-6736(93)92933-K"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116349"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1161\/atv91.13.9.8364014"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.17.5.873"},{"key":"e_1_3_2_33_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI114507"},{"key":"e_1_3_2_34_2","doi-asserted-by":"crossref","first-page":"1060","DOI":"10.1016\/S0022-2275(20)37920-7","volume":"24","year":"1983","journal-title":"J Lipid Res"},{"key":"e_1_3_2_35_2","first-page":"17","volume":"256","year":"1981","journal-title":"J Biol Chem"},{"key":"e_1_3_2_36_2","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/S0022-2275(20)37273-4","volume":"38","year":"1997","journal-title":"J Lipid Res"},{"key":"e_1_3_2_37_2","doi-asserted-by":"crossref","first-page":"1445","DOI":"10.1016\/S0022-2275(20)38265-1","volume":"30","year":"1989","journal-title":"J Lipid Res"},{"key":"e_1_3_2_38_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0026-0495(97)90267-5"},{"key":"e_1_3_2_39_2","doi-asserted-by":"publisher","DOI":"10.1161\/atvb.16.1.149"},{"key":"e_1_3_2_40_2","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1016\/S0022-2275(20)37424-1","volume":"38","year":"1997","journal-title":"J Lipid Res."},{"key":"e_1_3_2_41_2","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(86)28070-2"},{"key":"e_1_3_2_42_2","first-page":"E968","volume":"262","year":"1992","journal-title":"Am J Physiol"},{"key":"e_1_3_2_43_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00401a005"},{"key":"e_1_3_2_44_2","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/S0022-2275(20)34388-1","volume":"26","year":"1985","journal-title":"J Lipid Res"},{"key":"e_1_3_2_45_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113266"},{"key":"e_1_3_2_46_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI108761"},{"key":"e_1_3_2_47_2","doi-asserted-by":"publisher","DOI":"10.1016\/0925-4439(93)90149-U"},{"key":"e_1_3_2_48_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI116231"},{"key":"e_1_3_2_49_2","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/S0022-2275(20)41516-0","volume":"33","year":"1992","journal-title":"J Lipid Res"},{"key":"e_1_3_2_50_2","doi-asserted-by":"crossref","first-page":"2207","DOI":"10.1016\/S0022-2275(20)35361-X","volume":"34","year":"1993","journal-title":"J Lipid Res"}],"container-title":["Arteriosclerosis, Thrombosis, and Vascular Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/01.ATV.18.4.655","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,17]],"date-time":"2022-03-17T17:37:52Z","timestamp":1647538672000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/01.ATV.18.4.655"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1998,4]]},"references-count":50,"journal-issue":{"issue":"4","published-print":{"date-parts":[[1998,4]]}},"alternative-id":["10.1161\/01.ATV.18.4.655"],"URL":"https:\/\/doi.org\/10.1161\/01.atv.18.4.655","relation":{},"ISSN":["1079-5642","1524-4636"],"issn-type":[{"value":"1079-5642","type":"print"},{"value":"1524-4636","type":"electronic"}],"subject":[],"published":{"date-parts":[[1998,4]]}}}