PUBLICATION
Conserved functions of RNA-binding proteins in muscle
- Authors
- Nikonova, E., Kao, S.Y., Ravichandran, K., Wittner, A., Spletter, M.L.
- ID
- ZDB-PUB-190301-10
- Date
- 2019
- Source
- The international journal of biochemistry & cell biology 110: 29-49 (Journal)
- Registered Authors
- Keywords
- Drosophila, RNA-binding proteins, alternative splicing, development, muscle
- MeSH Terms
-
- Humans
- Neuromuscular Diseases/metabolism
- Muscles/metabolism*
- Animals
- RNA-Binding Proteins/genetics
- RNA-Binding Proteins/metabolism*
- Gene Expression Regulation
- PubMed
- 30818081 Full text @ Int. J. Biochem. Cell Biol.
Citation
Nikonova, E., Kao, S.Y., Ravichandran, K., Wittner, A., Spletter, M.L. (2019) Conserved functions of RNA-binding proteins in muscle. The international journal of biochemistry & cell biology. 110:29-49.
Abstract
Animals require different types of muscle for survival, for example for circulation, motility, reproduction and digestion. Much emphasis in the muscle field has been placed on understanding how transcriptional regulation generates diverse types of muscle during development. Recent work indicates that alternative splicing and RNA regulation are as critical to muscle development, and altered function of RNA-binding proteins causes muscle disease. Although hundreds of genes predicted to bind RNA are expressed in muscles, many fewer have been functionally characterized. We present a cross-species view summarizing what is known about RNA-binding protein function in muscle, from worms and flies to zebrafish, mice and humans. In particular, we focus on alternative splicing regulated by the CELF, MBNL and RBFOX families of proteins. We discuss the systemic nature of diseases associated with loss of RNA-binding proteins in muscle, focusing on mis-regulation of CELF and MBNL in myotonic dystrophy. These examples illustrate the conservation of RNA-binding protein function and the marked utility of genetic model systems in understanding mechanisms of RNA regulation.
Genes / Markers
Expression
Phenotype
Mutations / Transgenics
Human Disease / Model
Sequence Targeting Reagents
Fish
Orthology
Engineered Foreign Genes
Mapping