MKKS/BBS6 (chromosome 20p12.2) was the first gene coding a putative chaperonin to be associated with a human inherited disorder, the MKKS (Stone et al., 2000), being also involved in BBS shortly after (Katsanis et al., 2000; Slavotinek et al., 2000). Here, MKKS is linked to Bardet-Biedl syndrome.