{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,7]],"date-time":"2026-02-07T22:07:14Z","timestamp":1770502034234,"version":"3.49.0"},"reference-count":17,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2006,1,16]],"date-time":"2006-01-16T00:00:00Z","timestamp":1137369600000},"content-version":"vor","delay-in-days":411,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2004,12]]},"abstract":"<jats:p><jats:bold>A<jats:sc>bstract<\/jats:sc>: <\/jats:bold> Lysosome\u2010related organelles (LROs) are a family of cell\u2010type\u2010specific organelles that include melanosomes, platelet dense bodies, and cytotoxic T cell granules. The name, LRO, recognizes the fact that all of these organelles contain subsets of lysosomal proteins in addition to cell\u2010type\u2010specific proteins. The recent identification of genetic disorders that cause combined defects in several of these organelles indicates that they share common biogenetic pathways. Studies of one of these disorders, the Hermansky\u2010Pudlak syndrome (HPS), have provided helpful insights into the molecular machinery involved in LRO biogenesis. HPS is a genetically heterogeneous disorder caused by mutations in any of 7 genes in humans and 15 genes in mice. These genes encode subunits of 4 multi\u2010protein complexes named AP\u20103, BLOC\u20101, BLOC\u20102 and BLOC\u20103, in addition to miscellaneous components of the general protein trafficking machinery. The AP\u20103 complex is a coat protein involved in vesicle formation and cargo selection in the endosomal\u2010lysosomal system. One of these cargo molecules is the melanosomal enzyme, tyrosinase, the missorting of which may explain the defective melanosomes in AP\u20103\u2010deficient humans and mice. The function of the BLOC complexes is unknown, although they are thought to mediate either vesicle tethering\/fusion or cytoplasmic dispersal of LROs. Further studies of these complexes should contribute to the elucidation of the mechanisms of LRO biogenesis and the pathogenesis of HPS.<\/jats:p>","DOI":"10.1196\/annals.1315.018","type":"journal-article","created":{"date-parts":[[2005,4,18]],"date-time":"2005-04-18T23:52:53Z","timestamp":1113868373000},"page":"103-114","source":"Crossref","is-referenced-by-count":60,"title":["Insights into the Biogenesis of Lysosome\u2010Related Organelles from the Study of the Hermansky\u2010Pudlak Syndrome"],"prefix":"10.1111","volume":"1038","author":[{"given":"JUAN S.","family":"BONIFACINO","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2006,1,16]]},"reference":[{"key":"e_1_2_7_2_2","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.14.10.1265"},{"key":"e_1_2_7_3_2","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.biochem.72.121801.161800"},{"key":"e_1_2_7_4_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200307157"},{"key":"e_1_2_7_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0955-0674(00)00235-0"},{"key":"e_1_2_7_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/16.15.4508"},{"key":"e_1_2_7_7_2","doi-asserted-by":"publisher","DOI":"10.1007\/PL00008688"},{"key":"e_1_2_7_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s004380051099"},{"key":"e_1_2_7_9_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.137.4.835"},{"key":"e_1_2_7_10_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0749.2002.02074.x"},{"key":"e_1_2_7_11_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)80170-7"},{"key":"e_1_2_7_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/17.5.1304"},{"key":"e_1_2_7_13_2","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.12.7.2075"},{"key":"e_1_2_7_14_2","doi-asserted-by":"publisher","DOI":"10.1002\/bies.20042"},{"key":"e_1_2_7_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.ceb.2004.05.001"},{"key":"e_1_2_7_16_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.275.2.1300"},{"key":"e_1_2_7_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9343(99)00436-2"},{"key":"e_1_2_7_18_2","doi-asserted-by":"crossref","first-page":"1651","DOI":"10.1182\/blood.V99.5.1651.h8001651_1651_1658","article-title":"The Hermansky\u2010Pudlak syndrome 1 (HPS1) and HPS2 genes independently contribute to the production and function of platelet dense granules, melanosomes, and lysosomes","volume":"99","author":"Feng L.","year":"2002","journal-title":"Blood"}],"container-title":["Annals of the New York Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1196%2Fannals.1315.018","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/pdf\/10.1196\/annals.1315.018","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,25]],"date-time":"2023-09-25T19:25:55Z","timestamp":1695669955000},"score":1,"resource":{"primary":{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/10.1196\/annals.1315.018"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,12]]},"references-count":17,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2004,12]]}},"alternative-id":["10.1196\/annals.1315.018"],"URL":"https:\/\/doi.org\/10.1196\/annals.1315.018","archive":["Portico"],"relation":{},"ISSN":["0077-8923","1749-6632"],"issn-type":[{"value":"0077-8923","type":"print"},{"value":"1749-6632","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,12]]}}}