Physical and functional interaction of the ciliopathy proteins Lrrc56 and Odad3 control deployment of axonemal dyneins in vertebrate multiciliated cells.

ABSTRACT: Primary ciliary dyskinesia (PCD) is a genetically heterogeneous motile ciliopathy characterized by chronic respiratory disease, laterality defects, hydrocephalus and infertility, caused by impaired function of motile cilia. LRRC56 has recently emerged as a novel PCD candidate gene, but its role in vertebrate cilia remains poorly understood. Here, we used Xenopus laevis multiciliated cells, targeted knockdown and in vivo imaging to investigate lrrc56 function, and combined these studies with in vivo affinity purification-mass spectrometry (AP-MS) to define its interactome. We show that loss of lrrc56 causes specific depletion of outer dynein arms (ODAs) from the distal axoneme. In vivo AP-MS revealed that Lrrc56 binds the ODA docking complex components, including Odad3. Consistently, lrrc56

SUBMITTER: Reyes-Nava NG 

PROVIDER: S-EPMC12755065 | biostudies-literature | 2025 Dec

REPOSITORIES: biostudies-literature