Defects in BBS10 cause Bardet-Biedl syndrome 10 (BBS10) [MIM: 615987], an autosomal recessive ciliopathy characterized by retinitis pigmentosa, obesity, kidney dysfunction, polydactyly, obsessive-compulsive behavior, and hypogonadism [26]. Here, BBS10 is linked to Bardet-Biedl syndrome 10.