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Primary immunodeficiency or monogenic inflammatory bowel disease v9.16 BIRC3 Boaz Palterer gene: BIRC3 was added
gene: BIRC3 was added to Primary immunodeficiency or monogenic inflammatory bowel disease. Sources: Literature
Mode of inheritance for gene: BIRC3 was set to BOTH monoallelic and biallelic (but BIALLELIC mutations cause a more SEVERE disease form), autosomal or pseudoautosomal
Phenotypes for gene: BIRC3 were set to Inflammatory bowel disease; IBD; Crohn's disease
Penetrance for gene: BIRC3 were set to unknown
Review for gene: BIRC3 was set to GREEN
Added comment: Qi Li et al. described 14 patients from 10 unrelated families with monoallelic and biallelic variants in BIRC3 presenting with CD. Biallelic variants present more severe and earlier. Extensive funtional validation including zebrafish and mice models, recapitulating phenotype
https://www.gastrojournal.org/article/S0016-5085(26)06946-5/fulltext
Sources: Literature