{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T15:37:09Z","timestamp":1790005029819,"version":"4.0.1"},"reference-count":66,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2012,9,20]],"date-time":"2012-09-20T00:00:00Z","timestamp":1348099200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"crossref","award":["EY10199"],"award-info":[{"award-number":["EY10199"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"crossref"}]},{"name":"NHARP","award":["010019-0009-2009"],"award-info":[{"award-number":["010019-0009-2009"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Traffic"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:bold>Chediak\u2013Higashi syndrome (<jats:styled-content style=\"fixed-case\">CHS<\/jats:styled-content>) is a lethal disease caused by mutations that inactivate the lysosomal trafficking regulator protein (<jats:styled-content style=\"fixed-case\">LYST<\/jats:styled-content>). Patients suffer from diverse symptoms including oculocutaneous albinism, recurrent infections, neutropenia and progressive neurodegeneration. These defects have been traced back to over\u2010sized lysosomes and lysosome\u2010related organelles (<jats:styled-content style=\"fixed-case\">LROs<\/jats:styled-content>) in different cell types. Here, we explore mutants in the <jats:italic>Drosophila mauve<\/jats:italic> gene as a new model system for <jats:styled-content style=\"fixed-case\">CHS<\/jats:styled-content>. The <jats:italic>mauve<\/jats:italic> gene (<jats:styled-content style=\"fixed-case\">CG42863<\/jats:styled-content>) encodes a large <jats:styled-content style=\"fixed-case\">BEACH<\/jats:styled-content> domain protein of 3535 amino acids similar to <jats:styled-content style=\"fixed-case\">LYST<\/jats:styled-content>. This reflects a functional homology between these proteins as <jats:italic>mauve<\/jats:italic> mutants also display enlarged <jats:styled-content style=\"fixed-case\">LROs<\/jats:styled-content>, such as pigment granules. This <jats:italic>Drosophila<\/jats:italic> model also replicates the enhanced susceptibility to infections and we show a defect in the cellular immune response. Early stages of phagocytosis proceed normally in <jats:italic>mauve<\/jats:italic> mutant hemocytes but, unlike in wild type, late phagosomes fuse and generate large vacuoles containing many bacteria. Autophagy is similarly affected in <jats:italic>mauve<\/jats:italic> fat bodies as starvation\u2010induced autophagosomes grow beyond their normal size. Together these data suggest a model in which Mauve functions to restrict homotypic fusion of different pre\u2010lysosomal organelles and <jats:styled-content style=\"fixed-case\">LROs<\/jats:styled-content>.<\/jats:bold><\/jats:p>","DOI":"10.1111\/tra.12005","type":"journal-article","created":{"date-parts":[[2012,8,30]],"date-time":"2012-08-30T18:12:25Z","timestamp":1346350345000},"page":"1680-1692","source":"Crossref","is-referenced-by-count":44,"title":["<scp><i>Drosophila mauve<\/i><\/scp> Mutants Reveal a Role of <scp>LYST<\/scp> Homologs Late in the Maturation of Phagosomes and\u00a0Autophagosomes"],"prefix":"10.1111","volume":"13","author":[{"given":"Mokhlasur","family":"Rahman","sequence":"first","affiliation":[{"name":"Department of Neuroscience UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Haberman","sequence":"additional","affiliation":[{"name":"Department of Neuroscience UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles","family":"Tracy","sequence":"additional","affiliation":[{"name":"Department of Neuroscience UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanchali","family":"Ray","sequence":"additional","affiliation":[{"name":"Department of Neuroscience UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helmut","family":"Kr\u00e4mer","sequence":"additional","affiliation":[{"name":"Department of Neuroscience UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"},{"name":"Department of Cell Biology UT Southwestern Medical Center  5323 Harry Hines Blvd Dallas TX 75390\u20109111 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,9,20]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0037-1616221"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1023247215374"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/382262a0"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1196-307"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0796-303"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.genom.9.081307.164303"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1097\/MOH.0b013e3282f2bcce"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-197207000-00001"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(87)90020-1"},{"key":"e_1_2_6_11_1","first-page":"553","article-title":"Comparative studies of the Chediak\u2010Higashi syndrome","volume":"51","author":"Padgett GA","year":"1967","journal-title":"Am J Pathol"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1006\/mgme.1999.2927"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198204293061718"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1971.00490040015001"},{"key":"e_1_2_6_15_1","first-page":"156","article-title":"Neuropathological changes in Chediak\u2010Higashi disease","volume":"27","author":"Sung JH","year":"1968","journal-title":"J Neuropathol Exp Neurol"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.47.29790"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.4161\/auto.6.3.11226"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncomms1565"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/cdf502"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0854.2011.01300.x"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0854.2007.00567.x"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.2174\/1566524023362339"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0962-8924(98)01270-7"},{"key":"e_1_2_6_24_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.044230"},{"key":"e_1_2_6_25_1","doi-asserted-by":"publisher","DOI":"10.1139\/z88-191"},{"key":"e_1_2_6_26_1","first-page":"1277","volume-title":"The Development of Drosophila melanogaster","author":"Wolff T","year":"1993"},{"key":"e_1_2_6_27_1","volume-title":"The Genome of Drosophila melanogaster","author":"Lindsley DL","year":"1992"},{"key":"e_1_2_6_28_1","doi-asserted-by":"publisher","DOI":"10.1093\/bioinformatics\/btp151"},{"key":"e_1_2_6_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.10184"},{"key":"e_1_2_6_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2005.02.011"},{"key":"e_1_2_6_31_1","doi-asserted-by":"publisher","DOI":"10.1093\/molbev\/msr121"},{"key":"e_1_2_6_32_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201008119"},{"key":"e_1_2_6_33_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature02021"},{"key":"e_1_2_6_34_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.23.7.2501-2514.2003"},{"key":"e_1_2_6_35_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200412001"},{"key":"e_1_2_6_36_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.E08-03-0282"},{"key":"e_1_2_6_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1097-2765(00)80199-9"},{"key":"e_1_2_6_38_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200210166"},{"key":"e_1_2_6_39_1","doi-asserted-by":"publisher","DOI":"10.1101\/gad.1600707"},{"key":"e_1_2_6_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0076-6879(08)03235-7"},{"key":"e_1_2_6_41_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-59745-157-4_8"},{"key":"e_1_2_6_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2004.07.009"},{"key":"e_1_2_6_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2004.07.005"},{"key":"e_1_2_6_44_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201002035"},{"key":"e_1_2_6_45_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.01370"},{"key":"e_1_2_6_46_1","doi-asserted-by":"publisher","DOI":"10.1590\/S0074-02761992000400018"},{"key":"e_1_2_6_47_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197201202860302"},{"key":"e_1_2_6_48_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI106854"},{"key":"e_1_2_6_49_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.immunol.25.022106.141615"},{"key":"e_1_2_6_50_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpgi.00320.2011"},{"key":"e_1_2_6_51_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.01-09-0454"},{"key":"e_1_2_6_52_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1600-0854.2000.010508.x"},{"key":"e_1_2_6_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yexcr.2006.03.002"},{"key":"e_1_2_6_54_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.009001"},{"key":"e_1_2_6_55_1","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1242\/jcs.115.4.737","article-title":"Two members of the beige\/CHS (BEACH) family are involved at different stages in the organization of the endocytic pathway in Dictyostelium","volume":"115","author":"Cornillon S","year":"2002","journal-title":"J Cell Sci"},{"key":"e_1_2_6_56_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03402003"},{"key":"e_1_2_6_57_1","doi-asserted-by":"publisher","DOI":"10.1038\/nmeth.1331"},{"key":"e_1_2_6_58_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.02034"},{"key":"e_1_2_6_59_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.10.4.847"},{"key":"e_1_2_6_60_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.02502"},{"key":"e_1_2_6_61_1","doi-asserted-by":"publisher","DOI":"10.1038\/352207a0"},{"key":"e_1_2_6_62_1","doi-asserted-by":"publisher","DOI":"10.1091\/mbc.9.7.1709"},{"key":"e_1_2_6_63_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.133.6.1205"},{"key":"e_1_2_6_64_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1000377"},{"key":"e_1_2_6_65_1","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1242\/jcs.111.6.737","article-title":"Oligomerization of the extracellular domain of Boss enhances its binding to the Sevenless receptor and its antagonistic effect on R7 induction","volume":"111","author":"Sevrioukov EA","year":"1998","journal-title":"J Cell Sci"},{"key":"e_1_2_6_66_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/16.15.4508"},{"key":"e_1_2_6_67_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1558-5646.1985.tb00420.x"}],"container-title":["Traffic"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Ftra.12005","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/tra.12005","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T07:49:01Z","timestamp":1698652141000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/tra.12005"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,9,20]]},"references-count":66,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2012,12]]}},"alternative-id":["10.1111\/tra.12005"],"URL":"https:\/\/doi.org\/10.1111\/tra.12005","archive":["Portico"],"relation":{},"ISSN":["1398-9219","1600-0854"],"issn-type":[{"value":"1398-9219","type":"print"},{"value":"1600-0854","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,9,20]]}}}