Structure of the Decorated Ciliary Doublet Microtubule.
Cell
; 179(4): 909-922.e12, 2019 10 31.
Article
en En
| MEDLINE
| ID: mdl-31668805
ABSTRACT
The axoneme of motile cilia is the largest macromolecular machine of eukaryotic cells. In humans, impaired axoneme function causes a range of ciliopathies. Axoneme assembly, structure, and motility require a radially arranged set of doublet microtubules, each decorated in repeating patterns with non-tubulin components. We use single-particle cryo-electron microscopy to visualize and build an atomic model of the repeating structure of a native axonemal doublet microtubule, which reveals the identities, positions, repeat lengths, and interactions of 38 associated proteins, including 33 microtubule inner proteins (MIPs). The structure demonstrates how these proteins establish the unique architecture of doublet microtubules, maintain coherent periodicities along the axoneme, and stabilize the microtubules against the repeated mechanical stress induced by ciliary motility. Our work elucidates the architectural principles that underpin the assembly of this large, repetitive eukaryotic structure and provides a molecular basis for understanding the etiology of human ciliopathies.
Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Cilios
/
Axonema
/
Ciliopatías
/
Microtúbulos
Límite:
Humans
Idioma:
En
Año:
2019
Tipo del documento:
Article