Centriole biogenesis is seeded by CEP152-CEP63-PCNT aggregates propagating outside the centriole through the Alstrom syndrome protein ALMS1.

ABSTRACT: While subcellular structures like centrioles assemble flawlessly from protein components, it is unknown whether instructions from outside the components are involved. Centrioles build on the cartwheel scaffold that assembles in interphase and disassembles in mitosis following centriole growth, yet what underlies these cartwheel dynamics remains obscure. Here we identify ALMS1-an intrinsically disordered, disease-linked protein-as an external cofactor that preserves cartwheel-forming capacity without itself entering the cartwheel. Introducing disease-linked mutations disrupted cartwheel dynamics, enabling cartwheels to expand and shed from growing centrioles, and, in turn, form additional centrioles, leading to reciprocal amplification. Molecularly, we identified CEP152, CEP63, and PCNT as

SUBMITTER: Ozaki K 

PROVIDER: S-EPMC12262202 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature