BBSome-interacting protein 1 (BBIP1)

BBSome-interacting protein 1 (BBIP1, also known as BBS18 or BBSome-interacting protein of 10 kDa) is the smallest subunit in the core of the BBSome, an octameric protein complex essential for the formation and function of primary cilia in eukaryotic cells[1][4]. BBIP1 serves as a critical structural scaffold within the BBSome core complex, winding through tetratricopeptide repeat (TPR) domains of the subunits BBS4 and BBS8, and stabilizing their interactions[1][4]. The BBSome acts as a cargo adapter for targeting specific signaling receptors to the ciliary membrane, and disruption of BBIP1 impairs BBSome assembly, cilia formation, and stability, leading to Bardet–Biedl syndrome, a multisystemic genetic ciliopathy[1][4]. BBIP1 is not a direct therapeutic target, receptor, or enzyme; rather, it is a vital structural protein essential for BBSome integrity and thus proper ciliary signaling. There are currently no drugs known to specifically target BBIP1, nor is it used as a direct clinical biomarker or therapeutic target[1][4].

Other names

BBSome-interacting protein 1BBSome-interacting protein of 10 kDaBBIP1BBIP10BBS18UPF0604 proteinNCRNA00081bA348N5.3