ALMS1 stabilizes the proximal centriole to drive transition zone assembly and ciliogenesis.

Loss of ALMS1 in human cells shortens centrioles and causes frequent proximal-end breaks, impairing microtubule organization and transition zone assembly.

• Why it matters: Alstrøm syndrome is linked to ALMS1 mutations, but how ALMS1 supports cilia function was unclear, especially given its proximal centriole localization.
• What they did: The study examined ALMS1-deficient centrioles and Golgi-derived cargo trafficking, including transition zone transmembrane components, and assessed ciliogenesis and ciliary membrane composition.
• The result: In mice, ALMS1 disruption leads to progressive, age-dependent loss of cilia in retina, kidney, and brain, implying compromised ciliary assembly and maintenance.

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