De Freitas, S.; Riparbelli, M. G.; Callaini, G.; Laporte, M. H.; Durand, B.; Morel, V.

Centrioles are highly organised microtubular scaffolds which grow and mature progressively during successive cell cycles. Their molecular organisation is extensively characterized, yet the contribution of several components to centriole assembly, maturation or stability is incompletely understood. Here, using ultrastructure expansion microscopy and transmission electron microscopy, we show that ALMS1, the protein mutated in Alstrom syndrome, is required for proper centriole architecture. In absence of ALMS1, RPE1 cells exhibit shorter centrioles with defects in the microtubular wall, including broken or missing triplets or open B/C tubules. These structural defects arise after procentriole assembly. We show that ALMS1 loss selectively reduces the proximal region proteins CCDC77 and CEP44, leaving intact central and distal ones. ALMS1 is further required for the recruitment of the proximal CEP135 cap and the clearance of {delta}-tubulin/GCP2pool present at the procentriole base. Our findings thus identify ALMS1 as a key organiser of the centriole proximal domain and required for remodelling and stabilising the proximal end of centrioles during cell cycle progression.