Among BBSome components, kidney anomalies showed a low frequency in patients with mutations in BBS1, BBS4, or BBS8 and a high frequency in those with mutations in BBS2, BBS7, or BBS9. Patients harbouring variants in chaperonins (BBS10, BBS12 and BBS6) are more likely to develop severe CKD than patients with BBS1 mutations. The gene discussed is BBS9; the disease is chronic kidney disease.