{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T17:44:46Z","timestamp":1791308686223,"version":"4.3.1"},"reference-count":50,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Circulation Research"],"published-print":{"date-parts":[[2010,3,5]]},"abstract":"<jats:p>\n            <jats:bold>\n              <jats:italic>\n                <jats:underline>Rationale<\/jats:underline>\n                :\n              <\/jats:italic>\n            <\/jats:bold>\n            Loss of\n            <jats:italic>Tbx1<\/jats:italic>\n            and decrease of retinoic acid (RA) synthesis result in DiGeorge\/velocardiofacial syndrome (DGS\/VCFS)-like phenotypes in mouse models, including defects in septation of the outflow tract of the heart and anomalies of pharyngeal arch-derived structures including arteries of the head and neck, laryngeal-tracheal cartilage, and thymus\/parathyroid. Wild-type levels of T-box transcription factor (Tbx)1 and RA signaling are required for normal pharyngeal arch artery development. Recent studies have shown that reduction of RA or loss of\n            <jats:italic>Tbx1<\/jats:italic>\n            alters the contribution of second heart field (SHF) progenitor cells to the elongating heart tube.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>\n                <jats:underline>Objective<\/jats:underline>\n                :\n              <\/jats:italic>\n            <\/jats:bold>\n            Here we tested whether Tbx1 and the RA signaling pathway interact during the deployment of the SHF and formation of the mature aortic arch.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>\n                <jats:underline>Methods and Results<\/jats:underline>\n                :\n              <\/jats:italic>\n            <\/jats:bold>\n            Molecular markers of the SHF, neural crest and smooth muscle cells, were analyzed in\n            <jats:italic>Raldh2<\/jats:italic>\n            ;\n            <jats:italic>Tbx1<\/jats:italic>\n            compound heterozygous mutants. Our results revealed that the SHF and outflow tract develop normally in\n            <jats:italic>Raldh2<\/jats:italic>\n            <jats:sup>+\/\u2212<\/jats:sup>\n            ;\n            <jats:italic>Tbx1<\/jats:italic>\n            <jats:sup>+\/\u2212<\/jats:sup>\n            embryos. However, we found that decreased levels of RA accelerate the recovery from arterial growth delay observed in\n            <jats:italic>Tbx1<\/jats:italic>\n            <jats:sup>+\/\u2212<\/jats:sup>\n            mutant embryos. This compensation coincides with the differentiation of smooth muscle cells in the 4th pharyngeal arch arteries, and is associated with severity of neural crest cell migration defects observed in these mutants.\n          <\/jats:p>\n          <jats:p>\n            <jats:bold>\n              <jats:italic>\n                <jats:underline>Conclusions<\/jats:underline>\n                :\n              <\/jats:italic>\n            <\/jats:bold>\n            Our data suggest that differences in levels of embryonic RA may contribute to the variability in great artery anomalies observed in DGS\/VCFS patients.\n          <\/jats:p>","DOI":"10.1161\/circresaha.109.205732","type":"journal-article","created":{"date-parts":[[2010,1,29]],"date-time":"2010-01-29T03:34:22Z","timestamp":1264736062000},"page":"686-694","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":95,"title":["Decreased Levels of Embryonic Retinoic Acid Synthesis Accelerate Recovery From Arterial Growth Delay in a Mouse Model of DiGeorge Syndrome"],"prefix":"10.1161","volume":"106","author":[{"given":"Lucile","family":"Ryckebu\u0308sch","sequence":"first","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Bertrand","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karim","family":"Mesbah","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanny","family":"Bajolle","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karen","family":"Niederreither","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert G.","family":"Kelly","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, Universite\u0301 de la Me\u0301diterrane\u0301e, Campus de Luminy, Marseille, France; Service de Cardiologie Pe\u0301diatrique-M3C (F.B.), Ho\u0302pital Necker..."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ste\u0301phane","family":"Zaffran","sequence":"additional","affiliation":[{"name":"From the Institut National de la Sante\u0301 et de la Recherche Me\u0301dicale, Unite\u0301 Mixte de Recherche S910 (L.R., N.B., S.Z.), Universite\u0301 de la Me\u0301diterrane\u0301e, Faculte\u0301 de Me\u0301decine de Marseille, France; Developmental Biology Institute of Marseille-Luminy (L.R., N.B., K.M., R.G.K., S.Z.), Centre National de la Recherche Scientifique, Unite\u0301 Mixte de Recherche 6216, 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