CEP135 is a centrosomal protein that serves as a critical scaffolding component for centriole biogenesis and microtubule organization. The protein functions as a structural linker that directly connects the central cartwheel hub protein hSAS-6 to outer microtubules via its C-terminal and N-terminal domains, respectively 1. CEP135 is essential for proper centriole assembly, with depletion leading to abnormal centriole structures with altered microtubule triplet numbers and shortened centrioles 1. The protein also interacts with CPAP, another microcephaly-associated protein, and is required for CPAP-mediated centriole elongation 1. Additionally, CEP135 regulates centrosomal microtubule nucleation, with its levels being negatively controlled by the tumor suppressor LZTS2 2. In reproductive biology, CEP135 localizes to sperm centriolar structures and is crucial for spermatogenesis, with knockout mice displaying oligoasthenoteratozoospermia and complete male infertility due to defects in acrosome formation, flagellum development, and sperm morphology 3. Clinically, CEP135 mutations cause primary autosomal recessive microcephaly-8 and have been associated with microcephalic primordial dwarfism 4. The protein has also been implicated in cancer, where it may promote nasopharyngeal carcinoma progression through NF-κB pathway activation 5.