Centrosomal protein 85-like (CEP85L)

Centrosomal protein 85-like (CEP85L) is a centrosome-associated protein localized specifically to the pericentriolar material and maternal centriole, where it organizes the microtubule cytoskeleton and is essential for neuronal migration by regulating the activity and localization of CDK5, LIS1, and other proteins involved in brain development[1][2][3]. Pathogenic variants in CEP85L cause neuronal migration defects resulting in posterior-predominant lissencephaly, a severe neurodevelopmental disorder characterized by a smooth brain and impaired cortical architecture[1][2]. Beyond developmental disorders, CEP85L is also implicated in oncogenesis as a fusion partner with kinases like ROS1 or PDGFRβ, generating chimeric oncoproteins that drive cancer cell proliferation and may be actionable with kinase inhibitors in specific tumor contexts[3]. No direct pharmacologic targets are currently described for wild-type CEP85L, but its fusions represent emerging biomarkers and therapeutic targets in cancer.

Other names

Centrosomal protein of 85 kDa-likeC6orf204NY-BR-15bA57K17.2Serologically defined breast cancer antigen NY-BR-15LIS10centrosomal protein 85 likeserologically defined breast cancer antigen NY-BR-15

Not applicable for wild-type CEP85L; for fusion proteins, constitutive kinase activation (e.g., ROS1 or PDGFRβ fusions) drives tumor proliferation[3]