PUBLICATION
Nuclear Pores Regulate Muscle Development and Maintenance by Assembling a Localized Mef2C Complex
- Authors
- Raices, M., Bukata, L., Sakuma, S., Borlido, J., Hernandez, L.S., Hart, D.O., D'Angelo, M.A.
- ID
- ZDB-PUB-170913-8
- Date
- 2017
- Source
- Developmental Cell 41: 540-554.e7 (Journal)
- Registered Authors
- D'angelo, Maximiliano
- Keywords
- MEF2C, Nup210, gene expression, genome organization, myofiber, myogenesis, nuclear envelope, nuclear periphery, nuclear pore complexes, sarcomere
- MeSH Terms
-
- Animals
- Cell Differentiation
- Cell Nucleus/genetics
- Embryo, Nonmammalian/cytology*
- Embryo, Nonmammalian/metabolism
- MEF2 Transcription Factors/genetics
- MEF2 Transcription Factors/metabolism*
- Muscle Development/genetics*
- Nuclear Envelope/genetics
- Nuclear Pore/physiology*
- Nuclear Pore Complex Proteins/genetics
- Nuclear Pore Complex Proteins/metabolism*
- Zebrafish/growth & development
- Zebrafish/metabolism*
- Zebrafish Proteins/genetics
- Zebrafish Proteins/metabolism*
- PubMed
- 28586646 Full text @ Dev. Cell
Citation
Raices, M., Bukata, L., Sakuma, S., Borlido, J., Hernandez, L.S., Hart, D.O., D'Angelo, M.A. (2017) Nuclear Pores Regulate Muscle Development and Maintenance by Assembling a Localized Mef2C Complex. Developmental Cell. 41:540-554.e7.
Abstract
Nuclear pore complexes (NPCs) are multiprotein channels connecting the nucleus with the cytoplasm. NPCs have been shown to have tissue-specific composition, suggesting that their function can be specialized. However, the physiological roles of NPC composition changes and their impacts on cellular processes remain unclear. Here we show that the addition of the Nup210 nucleoporin to NPCs during myoblast differentiation results in assembly of an Mef2C transcriptional complex required for efficient expression of muscle structural genes and microRNAs. We show that this NPC-localized complex is essential for muscle growth, myofiber maturation, and muscle cell survival and that alterations in its activity result in muscle degeneration. Our findings suggest that NPCs regulate the activity of functional gene groups by acting as scaffolds that promote the local assembly of tissue-specific transcription complexes and show how nuclear pore composition changes can be exploited to regulate gene expression at the nuclear periphery.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping