Details

Autor(en) / Beteiligte

• Li, Cui
• Zheng, Yu
• Zheng, Yufang
• Xu, Zhiheng

Titel

SRPS associated protein WDR60 regulates the multipolar-to-bipolar transition of migrating neurons during cortical development

Ist Teil von

• Cell death & disease, 2021-01, Vol.12 (1), p.75-75, Article 75

Ort / Verlag

London: Nature Publishing Group UK

Erscheinungsjahr

2021

Quelle

EZB-FREE-00999 freely available EZB journals

Beschreibungen/Notizen

• Mutations of WD40 repeat domain 60 ( WDR60 ) have been identified in short-rib polydactyly syndromes (SRPS I–V), a group of lethal congenital disorders characterized by short ribs, polydactyly, and a range of extraskeletal phenotypes. However, the underlying mechanism is still unclear. Here, we report that WDR60 is essential for embryonic development and plays a critical role in the multipolar-bipolar transition and migration of newborn neurons during brain development. Mechanically, we found that WDR60 was located at the microtubule-organizing center to control microtubule organization and possibly, the trafficking of cellular components. Importantly, the migration defect caused by Wdr60 knockdown could be rescued by the stable form of α-Tubulin, α-Tubulin K40Q (an acetylation-mimicking mutant). These findings identified a non-cilia function of WDR60 and provided insight into its biological function, as well as the pathogenesis of WDR60 deficiency associated with SRPS.