{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T00:14:40Z","timestamp":1791159280575,"version":"4.1.0"},"reference-count":56,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation Research"],"published-print":{"date-parts":[[1999,2,5]]},"abstract":"<jats:p>\n            <jats:italic>Abstract<\/jats:italic>\n            \u2014DiGeorge syndrome (DGS) is a congenital disease characterized by defects in organs and tissues that depend on contributions by cell populations derived from neural crest for proper development. A number of candidate genes that lie within the q11 region of chromosome 22 commonly deleted in DGS patients have been identified. Orthologues of the DGS candidate gene\n            <jats:italic>HIRA<\/jats:italic>\n            are expressed in the neural crest and in neural crest\u2013derived tissues in both chick and mouse embryos. By exposing a portion of the premigratory chick neural crest to phosphorothioate end\u2013protected antisense oligonucleotides, ex ovo, followed by orthotopic backtransplantation to the untreated embryos, we have shown that the functional attenuation of\n            <jats:italic>cHIRA<\/jats:italic>\n            in the chick cardiac neural crest results in a significantly increased incidence of persistent truncus arteriosus, a phenotypic change characteristic of DGS, but does not affect the repatterning aortic arch arteries, the ventricular function, or the alignment of the outflow tract.\n          <\/jats:p>","DOI":"10.1161\/01.res.84.2.127","type":"journal-article","created":{"date-parts":[[2012,6,12]],"date-time":"2012-06-12T00:47:05Z","timestamp":1339462025000},"page":"127-135","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":84,"title":["HIRA, a DiGeorge Syndrome Candidate Gene, Is Required for Cardiac Outflow Tract Septation"],"prefix":"10.1161","volume":"84","author":[{"given":"Michael J.","family":"Farrell","sequence":"first","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harriett","family":"Stadt","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen T.","family":"Wallis","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Scambler","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R. Lester","family":"Hixon","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond","family":"Wolfe","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linda","family":"Leatherbury","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Margaret L.","family":"Kirby","sequence":"additional","affiliation":[{"name":"From the Developmental Biology Program, Institute of Molecular Medicine, Medical College of Georgia (M.J.F., H.S., K.T.W., R.L.H., R.W., L.L., M.L.K.), Augusta, Ga, and Institute of Child Health (P.S.), London, United Kingdom."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"e_1_3_2_1_2","doi-asserted-by":"publisher","DOI":"10.1161\/res.80.4.437"},{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.30.10.852"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1161\/res.77.2.211"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/5.5.633"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.12.2099"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.10.1577"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1006\/geno.1995.9982"},{"key":"e_1_3_2_8_2","first-page":"722","volume":"58","year":"1996","unstructured":"Pizzuti A, Novelli G, Mari A, Ratti A, Colosimo A, Amati F, Penso D, Sangiuolo F, Calabrese G, Palka G, Silani V, Gennarelli M, Mingarelli R, Scarlato G, Scambler P, Dallapiccola B. Human homologue sequences to the Drosophila dishevelled segment-polarity gene are deleted in the DiGeorge syndrome. Am J Hum Genet. 1996;58:722\u2013729.","journal-title":"Am J Hum Genet"},{"key":"e_1_3_2_9_2","first-page":"23","volume":"59","year":"1996","unstructured":"Sutherland HF, Wadey R, McKie JM, Taylor C, Atif U, Johnstone KA, Halford S, Kim U-J, Goodship J, Baldini A, Scambler PJ. Identification of a novel transcript disrupted by a balanced translocation associated with DiGeorge syndrome. Am J Hum Genet. 1996;59:23\u201331.","journal-title":"Am J Hum Genet"},{"key":"e_1_3_2_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.6.1027"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/2.10.1583"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.4.551"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/4.5.791"},{"key":"e_1_3_2_14_2","doi-asserted-by":"publisher","DOI":"10.1101\/gr.6.1.43"},{"key":"e_1_3_2_15_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.2.237"},{"key":"e_1_3_2_16_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.91.2.494"},{"key":"e_1_3_2_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/5.6.789"},{"key":"e_1_3_2_18_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90359-3"},{"key":"e_1_3_2_19_2","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(95)90358-5"},{"key":"e_1_3_2_20_2","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.51.32499"},{"key":"e_1_3_2_21_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.88.4.1516"},{"key":"e_1_3_2_22_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.275.5304.1301"},{"key":"e_1_3_2_23_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.8430321"},{"key":"e_1_3_2_24_2","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1993.1109"},{"key":"e_1_3_2_25_2","doi-asserted-by":"publisher","DOI":"10.1242\/dev.111.4.1173"},{"key":"e_1_3_2_26_2","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.130.4.997"},{"key":"e_1_3_2_27_2","doi-asserted-by":"publisher","DOI":"10.1242\/dev.122.4.1243"},{"key":"e_1_3_2_28_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0177(199701)208:1<34::AID-AJA4>3.0.CO;2-2"},{"key":"e_1_3_2_29_2","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1996.0243"},{"key":"e_1_3_2_30_2","doi-asserted-by":"publisher","DOI":"10.1161\/res.66.6.1693103"},{"key":"e_1_3_2_31_2","doi-asserted-by":"publisher","DOI":"10.1002\/aja.1001980407"},{"key":"e_1_3_2_32_2","doi-asserted-by":"publisher","DOI":"10.1038\/372333a0"},{"key":"e_1_3_2_33_2","doi-asserted-by":"publisher","DOI":"10.1203\/00006450-199112000-00002"},{"key":"e_1_3_2_34_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.84.3.1884453"},{"key":"e_1_3_2_35_2","unstructured":"Bharati S Lev M. The Pathology of Congenital Heart Disease. Armonk NY: Futura Publishing Co Inc; 1996:353\u2013380."},{"key":"e_1_3_2_36_2","unstructured":"Van Mierop LHS Kusche LM. Some aspects of comparative anatomy of the heart. In: Johansen K Burggren WW eds. Cardiovascular Shunts: Alfred Benzon Symposium 21. Copenhagen Denmark: Munksgaard; 1985:38\u201356."},{"key":"e_1_3_2_37_2","doi-asserted-by":"publisher","DOI":"10.1006\/excr.1996.0129"},{"key":"e_1_3_2_38_2","doi-asserted-by":"publisher","DOI":"10.1086\/515508"},{"key":"e_1_3_2_39_2","unstructured":"LeDouarin NM. The Neural Crest. Cambridge UK: Cambridge University Press; 1982."},{"key":"e_1_3_2_40_2","unstructured":"Horstadius S. The Neural Crest: Its Properties and Derivatives in the Light of Experimental Research. London UK: Oxford University Press; 1950."},{"key":"e_1_3_2_41_2","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-12-028608-9.50006-5"},{"key":"e_1_3_2_42_2","doi-asserted-by":"publisher","DOI":"10.1096\/fasebj.8.10.8050668"},{"key":"e_1_3_2_43_2","unstructured":"Johnston MC Sulik KK. The neural crest. 1995:278\u2013287."},{"key":"e_1_3_2_44_2","doi-asserted-by":"publisher","DOI":"10.1126\/science.6844926"},{"key":"e_1_3_2_45_2","doi-asserted-by":"publisher","DOI":"10.1006\/dbio.1998.8860"},{"key":"e_1_3_2_46_2","doi-asserted-by":"publisher","DOI":"10.1161\/circ.75.1.3791607"},{"key":"e_1_3_2_47_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.8.23.2857"},{"key":"e_1_3_2_48_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.92.8.3132"},{"key":"e_1_3_2_49_2","doi-asserted-by":"publisher","DOI":"10.1101\/gad.10.10.1247"},{"key":"e_1_3_2_50_2","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.18.9.5546"},{"key":"e_1_3_2_51_2","doi-asserted-by":"publisher","DOI":"10.1038\/1739"},{"key":"e_1_3_2_52_2","doi-asserted-by":"publisher","DOI":"10.1038\/380711a0"},{"key":"e_1_3_2_53_2","doi-asserted-by":"publisher","DOI":"10.1242\/dev.124.13.2659"},{"key":"e_1_3_2_54_2","first-page":"303","volume":"19","year":"1998","unstructured":"Ghyselinck NB, Wendling O, Messaddeq N, Dierich A, Lampron C, Decimo D, Viville S, Chambon P, Mark M. Contribution of retinoic acid receptor beta isoforms to the formation of the conotruncal septum of the embryonic heart. Dev Biol. 1998;19:303\u2013318.","journal-title":"Dev Biol"},{"key":"e_1_3_2_55_2","first-page":"289","volume":"194","year":"1997","unstructured":"M\u00e4nner J, Seidl W, Steding G. Experimental study on the significance of abnormal cardiac looping for the development of cardiovascular anomalies in neural crest-ablated chick embryos. Anat Embryol. 1997;194:289\u2013300.","journal-title":"Anat Embryol"},{"key":"e_1_3_2_56_2","doi-asserted-by":"publisher","DOI":"10.1161\/res.80.4.473"}],"container-title":["Circulation Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ahajournals.org\/doi\/full\/10.1161\/01.RES.84.2.127","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,5,12]],"date-time":"2024-05-12T20:57:42Z","timestamp":1715547462000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ahajournals.org\/doi\/10.1161\/01.RES.84.2.127"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1999,2,5]]},"references-count":56,"journal-issue":{"issue":"2","published-print":{"date-parts":[[1999,2,5]]}},"alternative-id":["10.1161\/01.RES.84.2.127"],"URL":"https:\/\/doi.org\/10.1161\/01.res.84.2.127","relation":{},"ISSN":["0009-7330","1524-4571"],"issn-type":[{"value":"0009-7330","type":"print"},{"value":"1524-4571","type":"electronic"}],"subject":[],"published":{"date-parts":[[1999,2,5]]},"assertion":[{"value":"1998-02-10","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"1998-10-23","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"1999-02-05","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}