Article

CCDC103-mediated assembly of the R2C complex links RUVBL1-RUVBL2 to Primary Ciliary Dyskinesia

2025-09-13

Abstract excerpt

Primary ciliary dyskinesia (PCD) is a genetic disorder caused by defective cilia motility, powered by axonemal dynein motors. Assembly of these motors is facilitated by the molecular chaperone HSP90, its co-chaperone RUVBL1-RUVBL2 and adaptor proteins such as CCDC103 (aka DNAAF19). Mutations in CCDC103 identified in PCD patients impair dynein assembly, contributing to the disease pathology. Here, we present the cr...

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Literature Corpus work: ebbbe6fa-10b3-54e9-857f-049185e3301b
DOI: 10.1101/2025.09.11.675549

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