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These results have evolutionary implications for studies of the drivers of negative selection, and practical implications in the search for genes underlying life-threatening, heritable conditions.</jats:p>","journal":"Proceedings of the National Academy of Sciences","year":2021,"id":600774,"datarank":0.6646225198264971,"base_score":4.430816798843313,"endowment":4.430816798843313,"self_citation_contribution":0.6646225198264971,"citation_network_contribution":0.0,"self_endowment_contribution":0.6646225198264971,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":83,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":241303,"name":"Bertrand Boisson","orcid":"0000-0001-5240-3555","position":1,"is_corresponding":false},{"id":383481,"name":"Anne Gregor","orcid":"0000-0001-9166-6593","position":2,"is_corresponding":false},{"id":104147,"name":"Vivien Béziat","orcid":"0000-0002-4020-824X","position":3,"is_corresponding":false},{"id":1540273,"name":"Stéphanie Boisson-Dupuis","orcid":null,"position":4,"is_corresponding":false},{"id":241304,"name":"Jacinta Bustamante","orcid":"0000-0002-3439-2482","position":5,"is_corresponding":false},{"id":104229,"name":"Emmanuelle Jouanguy","orcid":"0000-0002-7358-9157","position":6,"is_corresponding":false},{"id":104226,"name":"Anne Puel","orcid":"0000-0003-2603-0323","position":7,"is_corresponding":false},{"id":104146,"name":"Jérémie Rosain","orcid":"0000-0002-2822-161X","position":8,"is_corresponding":false},{"id":345059,"name":"Qian Zhang","orcid":"0009-0001-6172-4411","position":9,"is_corresponding":false},{"id":57374,"name":"Shen-Ying Zhang","orcid":"0000-0002-9449-3672","position":10,"is_corresponding":false},{"id":286444,"name":"Joseph G. 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We find pervasive negative selection at genes underlying both autosomal dominant and the most severe autosomal recessive inborn errors. Among genes underlying dominant disorders, we show that loss of function entails stronger selection than gain of function, and haploinsufficiency than negative dominance. We develop a method that predicts whether an autosomal gene is more likely to underlie a recessive or a dominant disease. 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