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0Recent scientific research uses in situ cryo-electron tomography to uncover the molecular composition of the transition zone (TZ) in human motile cilia, revealing nine specific constituent proteins. The study identifies ECT2L and DZANK1 as critical components that form the linker complexes connecting adjacent doublet microtubules. Genetic screening of individuals with suspected respiratory disorders linked biallelic loss-of-function variants in these newly identified linker genes to primary ciliary dyskinesia. Consequently, disruptions in the TZ architecture impair structural stability, compromise gating functions, and result in defective mucociliary transport.
References:
Zhou H, Terbeck L, Berical A, et al. In situ structure of the human ciliary transition zone links linker defects to primary ciliary dyskinesia[J]. Science, 2026: eaei5957.
