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U.S.A."],"published-print":{"date-parts":[[2023,4,18]]},"abstract":"<jats:p>\n                    While somatic variants of\u00a0\n                    <jats:italic>TRAF7<\/jats:italic>\n                    (Tumor necrosis factor\u00a0receptor-associated\u00a0factor\u00a07) underlie anterior skull-base meningiomas, here we report the inherited mutations of\u00a0\n                    <jats:italic>TRAF7<\/jats:italic>\n                    \u00a0that cause congenital heart defects. We show that TRAF7 mutants operate in a dominant manner, inhibiting protein function via heterodimerization with wild-type protein. Further, the shared genetics of the two disparate pathologies can be traced to the common origin of forebrain meninges and cardiac outflow tract from the\n                    <jats:italic>TRAF7-<\/jats:italic>\n                    expressing neural crest. Somatic and inherited mutations disrupt TRAF7\u2013IFT57 interactions leading to cilia degradation.\u00a0\n                    <jats:italic>TRAF7<\/jats:italic>\n                    -mutant meningioma primary cultures lack cilia, and TRAF7 knockdown causes cardiac, craniofacial, and ciliary defects in\u00a0\n                    <jats:italic>Xenopus<\/jats:italic>\n                    and zebrafish, suggesting a mechanistic convergence for\u00a0\n                    <jats:italic>TRAF7<\/jats:italic>\n                    -driven meningiomas and developmental heart defects.\n                  <\/jats:p>","DOI":"10.1073\/pnas.2214997120","type":"journal-article","created":{"date-parts":[[2023,4,12]],"date-time":"2023-04-12T13:44:51Z","timestamp":1681307091000},"update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":22,"title":["Pleiotropic role of TRAF7 in skull-base meningiomas and congenital heart 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