{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T04:30:37Z","timestamp":1791088237970,"version":"4.1.0"},"reference-count":55,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"10","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["ATVB"],"published-print":{"date-parts":[[1999,10]]},"abstract":"<jats:p>\n            <jats:italic>Abstract<\/jats:italic>\n            \u2014Anderson\u2019s disease is a rare, hereditary hypocholesterolemic syndrome characterized by chronic diarrhea, steatorrhea, and failure to thrive associated with the absence of apo B48\u2013containing lipoproteins. To further define the molecular basis of the disease, we studied 8 affected subjects in 7 unrelated families of North African origin after treatment with a low-fat diet. Lipid loading of intestinal biopsies persisted, but the pattern and extent of loading was variable among the patients. Electron microscopy showed lipoprotein-like particles in membrane-bound compartments, the densities (0.65 to 7.5 particles\/\u03bc\n            <jats:sup>2<\/jats:sup>\n            ) and the mean diameters (169 to 580 nm) of which were, in general, significantly larger than in a normal fed subject (0.66 particles\/\u03bc\n            <jats:sup>2<\/jats:sup>\n            , 209 nm mean diameter). There were also large lipid particles having diameters up to 7043 nm (average diameters from 368 to 2127 nm) that were not surrounded by a membrane. Rarely, lipoprotein-like particles 50 to 150 nm in diameter were observed in the intercellular spaces. Intestinal organ culture showed that apo B and apo AIV were synthesized with apparently normal molecular weights and that small amounts were secreted in lipid-bound forms (density &lt;1.006 g\/mL). Normal microsomal triglyceride transfer protein (MTP) and activity were also detected in intestinal biopsies. Segregation analyses of 4 families excluded, as a cause of the disease, significant regions of the genome surrounding the genes for apo AI, AIV, B, CI, CII, CIII, and E, as were the genes encoding 3 proteins involved in intracellular lipid transport, MTP, and fatty acid binding proteins 1 and 2. The results suggest that a factor other than apoproteins and MTP are important for human intestinal chylomicron assembly and secretion.\n          <\/jats:p>","DOI":"10.1161\/01.atv.19.10.2494","type":"journal-article","created":{"date-parts":[[2011,6,17]],"date-time":"2011-06-17T19:44:19Z","timestamp":1308339859000},"page":"2494-2508","source":"Crossref","is-referenced-by-count":56,"title":["Anderson\u2019s Disease"],"prefix":"10.1161","volume":"19","author":[{"given":"A. Hayssam","family":"Dannoura","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nathalie","family":"Berriot-Varoqueaux","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia","family":"Amati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Veronique","family":"Abadie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Verthier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jacques","family":"Schmitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R.","family":"Wetterau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie-Elisabeth","family":"Samson-Bouma","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence P.","family":"Aggerbeck","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","unstructured":"Kane JP Havel RJ. Disorders of the biogenesis and secretion of lipoproteins containing the B apolipoproteins. In: Scriver CR Beaudet AR Sly WS Valle D Stanbury JB Wyngaarden JB Frederickson DS eds. The Metabolic Basis of Inherited Disease . New York: McGraw-Hill;1989:1139\u20131164."},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1097\/00041433-199706000-00002"},{"key":"e_1_3_2_3_2","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1016\/S0022-2275(20)39976-4","volume":"34","year":"1993","journal-title":"J Lipid Res"},{"key":"e_1_3_2_4_2","first-page":"617","volume":"11","year":"1961","journal-title":"Med J Aust"},{"key":"e_1_3_2_5_2","first-page":"1079","volume":"24","year":"1967","journal-title":"Arch Fr Pe\u0301diatr"},{"key":"e_1_3_2_6_2","unstructured":"Silverberg M Kessler J Neumann PZ Wiglesworth FW. An intestinal lipid transport defect. A possible variant of hypo-beta-lipoproteinemia. Gastroenterolog y. 1968;54:1271. Abstract."},{"key":"e_1_3_2_7_2","first-page":"342","volume":"17","year":"1970","journal-title":"Ann Pediatr (Paris)"},{"key":"e_1_3_2_8_2","first-page":"1022","volume":"58","year":"1970","journal-title":"Gastroenterology"},{"key":"e_1_3_2_9_2","unstructured":"Costil J. Maladie d\u2019Anderson. In: Journe\u0301es Parisiennes de Pediatrie . Paris: Flammarion; 1976:229\u2013239."},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1136\/gut.20.2.163"},{"key":"e_1_3_2_11_2","first-page":"591","volume":"14","year":"1983","journal-title":"Ann Neurol"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI112590"},{"key":"e_1_3_2_13_2","first-page":"185","volume":"24","year":"1986","journal-title":"An Esp Pediatr"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(87)90133-8"},{"key":"e_1_3_2_15_2","doi-asserted-by":"crossref","first-page":"1263","DOI":"10.1016\/S0022-2275(20)38593-X","volume":"28","year":"1987","journal-title":"J Lipid Res"},{"key":"e_1_3_2_16_2","first-page":"491","volume":"46","year":"1989","journal-title":"Arch Fr Pe\u0301diatr"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI114996"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199304000-00006"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0021-9150(94)90115-5"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2362.1995.tb01559.x"},{"key":"e_1_3_2_21_2","first-page":"195","volume":"47","year":"1997","journal-title":"An Esp Pediatr"},{"key":"e_1_3_2_22_2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0022-2275(20)42755-5","volume":"31","year":"1990","journal-title":"J Lipid Res"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.1439810"},{"key":"e_1_3_2_24_2","doi-asserted-by":"crossref","first-page":"19709","DOI":"10.1016\/S0021-9258(19)77693-5","volume":"263","year":"1988","journal-title":"J Biol Chem"},{"key":"e_1_3_2_25_2","doi-asserted-by":"crossref","first-page":"7242","DOI":"10.1016\/S0021-9258(18)68634-X","volume":"263","year":"1988","journal-title":"J Biol Chem"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI113646"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI103182"},{"key":"e_1_3_2_28_2","unstructured":"Sambrook J Fritsch EF Maniatis T. Molecular cloning. A laboratory manual . Cold Spring Harbor Laboratory Press; 1989."},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00197a005"},{"key":"e_1_3_2_30_2","first-page":"4299","volume":"18","year":"1990","journal-title":"Nucl Acids Res"},{"key":"e_1_3_2_31_2","first-page":"388","volume":"44","year":"1989","journal-title":"Am J Hum Genet"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1093\/nar\/19.17.4799"},{"key":"e_1_3_2_33_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.93.25.14771"},{"key":"e_1_3_2_34_2","doi-asserted-by":"publisher","DOI":"10.1038\/380152a0"},{"key":"e_1_3_2_35_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.81.11.3443"},{"key":"e_1_3_2_36_2","unstructured":"Ott J. Analysis of human genetic linkage . Baltimore: Johns Hopkins University Press; 1991."},{"key":"e_1_3_2_37_2","unstructured":"Terwilliger JD Ott J. Handbook of human genetic linkage . Baltimore: Johns Hopkins University Press; 1994."},{"key":"e_1_3_2_38_2","first-page":"277","volume":"7","year":"1955","journal-title":"Am J Hum Genet"},{"key":"e_1_3_2_39_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.1984.tb00849.x"},{"key":"e_1_3_2_40_2","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1016\/S0022-2275(20)32183-0","volume":"39","year":"1998","journal-title":"J Lipid Res"},{"key":"e_1_3_2_41_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI118365"},{"key":"e_1_3_2_42_2","doi-asserted-by":"publisher","DOI":"10.1042\/bj2470053"},{"key":"e_1_3_2_43_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1768-322X.1996.tb00980.x"},{"key":"e_1_3_2_44_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00337a028"},{"key":"e_1_3_2_45_2","doi-asserted-by":"crossref","first-page":"1998","DOI":"10.1016\/S0021-9258(17)35888-X","volume":"261","year":"1986","journal-title":"J Biol Chem"},{"key":"e_1_3_2_46_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00453a029"},{"key":"e_1_3_2_47_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi00361a034"},{"key":"e_1_3_2_48_2","doi-asserted-by":"crossref","first-page":"677","DOI":"10.1016\/S0016-5085(19)31679-8","volume":"75","year":"1978","journal-title":"Gastroenterology"},{"key":"e_1_3_2_49_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.86.2.562"},{"key":"e_1_3_2_50_2","doi-asserted-by":"crossref","first-page":"8612","DOI":"10.1016\/S0021-9258(18)43542-9","volume":"255","year":"1980","journal-title":"J Biol Chem"},{"key":"e_1_3_2_51_2","doi-asserted-by":"crossref","first-page":"1223","DOI":"10.1016\/S0016-5085(82)80132-7","volume":"83","year":"1982","journal-title":"Gastroenterology"},{"key":"e_1_3_2_52_2","doi-asserted-by":"publisher","DOI":"10.1016\/0163-7827(95)00005-K"},{"key":"e_1_3_2_53_2","doi-asserted-by":"publisher","DOI":"10.1172\/JCI119841"},{"key":"e_1_3_2_54_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.47.29945"},{"key":"e_1_3_2_55_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199502000-00002"}],"container-title":["Arteriosclerosis, Thrombosis, and Vascular Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/01.ATV.19.10.2494","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,17]],"date-time":"2022-03-17T14:42:55Z","timestamp":1647528175000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/01.ATV.19.10.2494"}},"subtitle":["Exclusion of Apolipoprotein and Intracellular Lipid Transport Genes"],"short-title":[],"issued":{"date-parts":[[1999,10]]},"references-count":55,"journal-issue":{"issue":"10","published-print":{"date-parts":[[1999,10]]}},"alternative-id":["10.1161\/01.ATV.19.10.2494"],"URL":"https:\/\/doi.org\/10.1161\/01.atv.19.10.2494","relation":{},"ISSN":["1079-5642","1524-4636"],"issn-type":[{"value":"1079-5642","type":"print"},{"value":"1524-4636","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,10]]}}}