Centrosomal protein of 97 kDa (CEP97)

Centrosomal protein of 97 kDa (CEP97) is a conserved centriolar protein localized at the distal end of the mother centriole. It acts as a key negative regulator of ciliogenesis by forming a complex with CP110 and capping the centriole, thereby preventing inappropriate cilia formation in cycling cells[4][5]. CEP97 is essential for maintaining centriole structural integrity and proper size; its absence leads to defective centrioles and impaired cilia biogenesis[1][2]. It plays a structural, non-enzymatic role linked to protein complexes involved in centriole stability, and it interacts with the microtubule-modifying enzyme SIRT2. Mutations in CEP97 are associated with rare developmental syndromes such as Seckel syndrome[5]. CEP97 is not currently regarded as a therapeutic target, and there are no known drugs that specifically target it.

Other names

Centrosomal protein 97CEP97LRRIQ2FLJ23047Leucine-rich repeat and IQ domain-containing protein 22810403B08RikLeucine-rich repeats and IQ motif containing 2