CEP170 (centrosomal protein 170) is a multifunctional centrosomal protein with critical roles in microtubule organization and cilia biology. Structurally, CEP170 localizes to centrosomes, centriolar subdistal appendages, and ciliary basal bodies, where it plays essential roles in centriole assembly and spindle organization 12. CEP170 promotes microtubule organization through interactions with regulatory proteins; Ccdc61 controls its centrosomal localization and regulates its binding to TANK binding kinase 1, which stabilizes microtubules 3. During cilia disassembly, CEP170 functions downstream of PI3K signaling via a PDK1/PKCι kinase axis and interacts with the KIF2A microtubule-depolymerizing kinesin 4. In dynein-2 assembly, CEP170 interacts with dynein-2 complexes to support intraflagellar transport and cilia function 5. CEP170 is essential for proper mitotic progression and spindle orientation; its knockdown causes spindle misorientation and impairs astral microtubule stability while affecting dynein/dynactin motor complex localization 6. Disease relevance includes associations with chr1 kidney disease as a validated causal protein 7 and esophageal cancer, where METTL3-mediated m6A modifications regulate CEP170 expression, promoting cell proliferation 6. CEP170 loss impairs cilia formation and hedgehog signaling 5, contributing to ciliopathy-like phenotypes 4.
No tissue expression data available for this gene.