These include the BBSome complex (Figure 1), a stable heptameric structure consisting of eight subunits, namely, the Bardet Biedl Syndrome (BBS)-associated proteins and interacting proteins (BBS1, BBS2, BBS4, BBS5, BBS7, BBS8, BBS9 and BBIP10/BBS18/BBIP1) [25,26,27]. This evidence concerns the gene BBS2 and Bardet-Biedl syndrome.