Inversin (INVS)

Inversin (INVS) is a ciliary protein with multiple ankyrin-repeat domains and IQ calmodulin-binding domains[2][4]. It is essential for normal renal development, particularly the development and maintenance of renal tubules, and plays a central role in the establishment of left-right asymmetry during embryonic development[2][3][4]. Inversin is considered the molecular switch between the canonical and non-canonical Wnt signaling pathways, inhibiting canonical Wnt signaling by targeting Dishevelled (DVL1) for proteasomal degradation[3][4]. This regulatory role underlies its importance in terminal differentiation of renal tubular epithelial cells and correct tissue organization[4]. INVS is a core component of the “inversin compartment” of the primary cilium, where it functions with ANKS6, NEK8, and NPHP3[1]. It also interacts with several proteins, including calmodulin, nephrocystin (NPHP1), Dishevelled (DVL), Akt, and beta-catenin[2][3]. Mutations in INVS cause nephronophthisis type 2, an autosomal recessive childhood-onset cystic kidney disease[2][4]. INVS is not classified as a typical therapeutic target (such as a receptor, enzyme, transporter, or ion channel), and no approved drugs are known to act directly on this protein.

Other names

INVNPHP2NPH2inversion of embryo turning homolognephrocystin-2nephrocystin 2inversin