{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T09:57:05Z","timestamp":1789984625164,"version":"4.0.1"},"reference-count":90,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2010,9,15]],"date-time":"2010-09-15T00:00:00Z","timestamp":1284508800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NICHD","award":["HD42182"],"award-info":[{"award-number":["HD42182"]}]},{"name":"NIMH Silvio M. Conte Grant","award":["MH64065"],"award-info":[{"award-number":["MH64065"]}]},{"name":"NARSAD Independent Investigator Awards"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Intl J of Devlp Neuroscience"],"published-print":{"date-parts":[[2011,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>DiGeorge, or 22q11 deletion syndrome (22q11DS), the most common survivable human genetic deletion disorder, is caused by deletion of a minimum of 32 contiguous genes on human chromosome 22, and presumably results from diminished dosage of one, some, or all of these genes\u2014particularly during development. Nevertheless, the normal functions of 22q11 genes in the embryo or neonate, and their contribution to developmental pathogenesis that must underlie 22q11DS are not well understood. Our data suggests that a substantial number of 22q11 genes act specifically and in concert to mediate early morphogenetic interactions and subsequent cellular differentiation at phenotypically compromised sites\u2014the limbs, heart, face and forebrain. When dosage of a broad set of these genes is diminished, early morphogenesis is altered, and initial 22q11DS phenotypes are established. Thereafter, functionally similar subsets of 22q11 genes\u2014especially those that influence the cell cycle or mitochondrial function\u2014remain expressed, particularly in the developing cerebral cortex, to regulate neurogenesis and synaptic development. When dosage of these genes is diminished, numbers, placement and connectivity of neurons and circuits essential for normal behavior may be disrupted. Such disruptions likely contribute to vulnerability for schizophrenia, autism, or attention deficit\/hyperactivity disorder seen in most 22q11DS patients.<\/jats:p>","DOI":"10.1016\/j.ijdevneu.2010.08.005","type":"journal-article","created":{"date-parts":[[2010,9,16]],"date-time":"2010-09-16T08:56:02Z","timestamp":1284627362000},"page":"283-294","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":55,"title":["Three phases of DiGeorge\/22q11 deletion syndrome pathogenesis during brain development: Patterning, proliferation, and mitochondrial functions of 22q11 genes"],"prefix":"10.1002","volume":"29","author":[{"given":"D.W.","family":"Meechan","sequence":"first","affiliation":[{"name":"Department of Pharmacology and Physiology and GW Institute for Neuroscience The George Washington University School of Medicine and Health Sciences 2300 Eye Street NW Washington DC United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.M.","family":"Maynard","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology and GW Institute for Neuroscience The George Washington University School of Medicine and Health Sciences 2300 Eye Street NW Washington DC United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.S.","family":"Tucker","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology and GW Institute for Neuroscience The George Washington University School of Medicine and Health Sciences 2300 Eye Street NW Washington DC United States"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.\u2010S.","family":"LaMantia","sequence":"additional","affiliation":[{"name":"Department of Pharmacology and Physiology and GW Institute for Neuroscience The George Washington University School of Medicine and Health Sciences 2300 Eye Street NW Washington DC United States"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,9,15]]},"reference":[{"issue":"8","key":"e_1_2_7_2_1","first-page":"7","article-title":"Fundamentals of attention\u2010deficit\/hyperactivity disorder: circuits and pathways","volume":"67","author":"Arnsten A.F.","year":"2006","journal-title":"J. Clin. Psychiatry"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.00581"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/cercor\/bhl097"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/cercor\/bhn064"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-3223(02)01360-4"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20041090"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-0173(99)00041-7"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1186\/1749-8104-3-17"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.1990.01810140081011"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1037\/0735-7044.115.4.957"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1086\/515508"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddh322"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0702225104"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.903410109"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.physiol.60.1.267"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.58.1.64"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0307-10.2010"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.129.19.4591"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.2001.0283"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.conb.2007.01.009"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/7777"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsycho.2007.11.009"},{"issue":"1","key":"e_1_2_7_24_1","doi-asserted-by":"crossref","first-page":"277","DOI":"10.1042\/bj3250277","article-title":"Expression of the murine RanBP1 and Htf9\u2010c genes is regulated from a shared bidirectional promoter during cell cycle progression","volume":"325","author":"Guarguaglini G.","year":"1997","journal-title":"Biochem. J."},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00523.2006"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.23-15-06315.2003"},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1096\/fj.08-127399"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-8993(79)90349-4"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.plipres.2009.02.001"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.270.29.17442"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.3844-09.2009"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroscience.2007.12.020"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pgen.1000252"},{"key":"e_1_2_7_34_1","doi-asserted-by":"publisher","DOI":"10.1093\/cercor\/bhn066"},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0079-6123(08)63623-7"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-3223(99)00002-5"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19961111)65:4<309::AID-AJMG12>3.0.CO;2-Y"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrn1648"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1038\/35065105"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.232186099"},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M206573200"},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-006-0054-0"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2008.12.044"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0925-4773(01)00616-5"},{"key":"e_1_2_7_45_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2235651100"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00335-006-0011-0"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2008.07.027"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.57.7.637"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2006.09.001"},{"key":"e_1_2_7_50_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0905696106"},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0092-8674(01)00247-1"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.64.7.945"},{"key":"e_1_2_7_53_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tig.2005.06.008"},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0602400103"},{"key":"e_1_2_7_55_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng1375"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn.2204"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.56.10.940"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0165-3806(00)00025-0"},{"key":"e_1_2_7_59_1","first-page":"185","article-title":"Mental retardation and behavior problems in 100 individuals with 22q11 deletion syndrome","volume":"31","author":"Niklasson L.","year":"2008","journal-title":"Res. Dev. Disabil."},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1038\/nn1172"},{"key":"e_1_2_7_61_1","doi-asserted-by":"publisher","DOI":"10.1101\/gr.206901"},{"key":"e_1_2_7_62_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0600206103"},{"key":"e_1_2_7_63_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.22.1.378-387.2002"},{"key":"e_1_2_7_64_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00738.2007"},{"key":"e_1_2_7_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-291X(80)91100-6"},{"key":"e_1_2_7_66_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.3952506"},{"key":"e_1_2_7_67_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.34.10.798"},{"key":"e_1_2_7_68_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2009.06.006"},{"key":"e_1_2_7_69_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.02297"},{"key":"e_1_2_7_70_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0679-08.2008"},{"key":"e_1_2_7_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pscychresns.2005.10.002"},{"key":"e_1_2_7_72_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.125.16.3111"},{"key":"e_1_2_7_73_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.mop.0000184465.73833.0b"},{"key":"e_1_2_7_74_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature08855"},{"key":"e_1_2_7_75_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2005.03257.x"},{"key":"e_1_2_7_76_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.138"},{"key":"e_1_2_7_77_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.02324"},{"key":"e_1_2_7_78_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2007.10.074"},{"key":"e_1_2_7_79_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddp086"},{"key":"e_1_2_7_80_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M504113200"},{"key":"e_1_2_7_81_1","doi-asserted-by":"publisher","DOI":"10.1002\/cne.10905"},{"key":"e_1_2_7_82_1","doi-asserted-by":"publisher","DOI":"10.1242\/jcs.009308"},{"key":"e_1_2_7_83_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.cb.10.110194.001005"},{"key":"e_1_2_7_84_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2341-08.2008"},{"key":"e_1_2_7_85_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpendo.00095.2006"},{"key":"e_1_2_7_86_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.61.11.1085"},{"key":"e_1_2_7_87_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0437920100"},{"key":"e_1_2_7_88_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ydbio.2005.04.029"},{"key":"e_1_2_7_89_1","doi-asserted-by":"publisher","DOI":"10.1001\/archpsyc.1987.01800190080012"},{"key":"e_1_2_7_90_1","doi-asserted-by":"publisher","DOI":"10.1016\/0893-133X(95)00199-N"},{"key":"e_1_2_7_91_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M209760200"}],"container-title":["International Journal of Developmental Neuroscience"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0736574810003783?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0736574810003783?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1016%2Fj.ijdevneu.2010.08.005","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.ijdevneu.2010.08.005","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1016\/j.ijdevneu.2010.08.005","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1016\/j.ijdevneu.2010.08.005","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,14]],"date-time":"2024-05-14T07:50:41Z","timestamp":1715673041000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1016\/j.ijdevneu.2010.08.005"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,9,15]]},"references-count":90,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2011,5]]}},"alternative-id":["10.1016\/j.ijdevneu.2010.08.005"],"URL":"https:\/\/doi.org\/10.1016\/j.ijdevneu.2010.08.005","archive":["Portico"],"relation":{},"ISSN":["0736-5748","1873-474X"],"issn-type":[{"value":"0736-5748","type":"print"},{"value":"1873-474X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,9,15]]},"assertion":[{"value":"2010-06-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2010-08-30","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2010-09-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}