4.7 Article

Lamin A-mediated nuclear lamina integrity is required for proper ciliogenesis

Journal

EMBO REPORTS
Volume 21, Issue 10, Pages -

Publisher

WILEY
DOI: 10.15252/embr.201949680

Keywords

cilia; ciliogenesis; ciliopathy; lamin; laminopathy

Funding

  1. Ministry of Science and Technology, Taiwan [106-2320-B-005-011-MY3, 107-2923-B-005-002-MY3, 108-2320-B-010-015-MY3]
  2. Cancer Progression Research Center, National Yang-Ming University from the Featured Areas Research Center Program within Ministry of Education (MOE) in Taiwan

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The primary cilium is a sensory organelle that receives specific signals from the extracellular environment important for vertebrate development and tissue homeostasis. Lamins, the major components of the nuclear lamina, are required to maintain the nuclear structure and are involved in most nuclear activities. In this study, we show that deficiency in lamin A/C causes defective ciliogenesis, accompanied by increased cytoplasmic accumulation of actin monomers and increased formation of actin filaments. Disruption of actin filaments by cytochalasin D rescues the defective ciliogenesis in lamin A/C-depleted cells. Moreover, lamin A/C-deficient cells display lower levels of nesprin 2 and defects in recruiting Arp2, myosin Va, and tau tubulin kinase 2 to the basal body during ciliogenesis. Collectively, our results uncover a functional link between nuclear lamina integrity and ciliogenesis and implicate the malfunction of primary cilia in the pathogenesis of laminopathy.

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