Rogdi atypical leucine zipper (ROGDI)

Rogdi atypical leucine zipper is a highly conserved, monomeric leucine zipper-like protein, present in metazoan organisms, encoded by the ROGDI gene on human chromosome 16[1][2][3][4][5]. Its structure comprises a unique curved architecture with a core four-helix bundle and a β-sheet domain, forming a leucine zipper-like but functionally and structurally distinct motif from classical coiled-coil ZIP proteins[1]. It is expressed at highest levels in the brain and spinal cord and is implicated in the development of the nervous system and teeth[5][3]. Mutations in ROGDI cause Kohlschutter-Tonz syndrome, a rare autosomal recessive disorder featuring epilepsy, spasticity, global developmental delay, and amelogenesis imperfecta (defective tooth enamel)[1][2][3][5]. Rogdi interacts with other neuronal proteins, including DISC1, suggesting a role for protein scaffolding in neurogenesis[1]. Despite its structural characterization, there is no evidence that Rogdi is a conventional therapeutic target such as a receptor, enzyme, ion channel, or transporter, and no drugs are currently known to act on Rogdi[1][2].

Other names

RogdiROGDI proteinLeucine zipper protein rogdi