Two previous studies identified more severe kidney disease associated with BBS2, BBS10, and BBS12 in a United Kingdom cohort of children and adults and BBS6, BBS10, and BBS12 in a French cohort of adults11, 13 Utilizing the largest international natural history registry, our findings provide critical insight and advance opportunities to explore the proteomics and metabolomics in a model renal ciliopathy.6, 15. This evidence concerns the gene BBS2 and kidney disorder.