@alanbrownhms.bsky.social
Structural biologist studying cilia and ciliopathies.
@alanbrownhms.bsky.socialLooks good! Would you mind attaching the supplement to the preprint? It would be nice to see the additional 33 figures!
@alanbrownhms.bsky.socialLed by Haixia Zhou and Lea Terbeck, and only possible through international collaboration with the Omran, Hawkins, and @centriolelab.bsky.social labs. We thank our funding agencies, everyone who helped with this project, and the families who took part.
@alanbrownhms.bsky.socialTEM, SEM, and high-speed videos showed that when either protein is lost, TZ architecture collapses, cilia develop swollen tips, and mucus transport stops. Deleting ECT2L in stem-cell-derived airway cells did the same. We think this is the first demonstration that TZ defects can cause PCD.
@alanbrownhms.bsky.socialReasoning that these linkers might matter for respiratory health, we teamed up with Heymut Omran's lab to screen 489 people with suspected primary ciliary dyskinesia (PCD) – an inherited respiratory disease – and found four families with variants in ECT2L or DZANK1
@alanbrownhms.bsky.socialFocusing on TZ doublet microtubules, our subtomogram averages showed that neighboring doublets are bridged by an unexpected set of proteins: ECT2L, DZANK1, SKP1, and DYNLL1/2
@alanbrownhms.bsky.socialMillions of beating cilia keep airways clean. At the base of each is the transition zone (TZ), a gate that controls which proteins enter and leave. In our paper out today in Science, we used cryo-FIB-ET to image the TZ inside human airway cells www.science.org/doi/10.1126/...