ArticleJournal of structural biology2010
Differential splicing of the large sarcomeric protein nebulin during skeletal muscle development.
Article in Journal of structural biology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
What it found
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Who cites it
24 citing papers in PubMed, 36 citations in OpenAlex.
- NRAP reduction rescues sarcomere defects in nebulin-related nemaline myopathy.Human molecular genetics · 2023Article
- Alternative splicing diversifies the skeletal muscle transcriptome during prolonged spaceflight.Skeletal muscle · 2022Article
- The Mechanisms of Thin Filament Assembly and Length Regulation in Muscles.International journal of molecular sciences · 2022Review
- A Homozygous Deep Intronic Mutation Alters the Splicing of Nebulin Gene in a Patient With Nemaline Myopathy.Frontiers in neurology · 2021Article
- Nebulin and Lmod2 are critical for specifying thin-filament length in skeletal muscle.Science advances · 2020Article
- A long-read RNA-seq approach to identify novel transcripts of very large genes.Genome research · 2020Article
- Nebulin: big protein with big responsibilities.Journal of muscle research and cell motility · 2020Review
- Expressing a Z-disk nebulin fragment in nebulin-deficient mouse muscle: effects on muscle structure and function.Skeletal muscle · 2020Article
- Deleting Titin's C-Terminal PEVK Exons Increases Passive Stiffness, Alters Splicing, and Induces Cross-Sectional and Longitudinal Hypertrophy in Skeletal Muscle.Frontiers in physiology · 2020Article
- Compliant Titin Isoform Content Is Reduced in Left Ventricles of Sedentary Versus Active Rats.Frontiers in physiology · 2020Article
- Two alternatively-spliced human nebulin isoforms with either exon 143 or exon 144 and their developmental regulation.Scientific reports · 2018Article
- Article
- Aberrant developmental titin splicing and dysregulated sarcomere length in Thymosin β4 knockout mice.Journal of molecular and cellular cardiology · 2017Article
- Nebulin, a multi-functional giant.The Journal of experimental biology · 2016Review
- Removal of immunoglobulin-like domains from titin's spring segment alters titin splicing in mouse skeletal muscle and causes myopathy.The Journal of general physiology · 2014Article
- Deleting exon 55 from the nebulin gene induces severe muscle weakness in a mouse model for nemaline myopathy.Brain : a journal of neurology · 2013Article
- A two-segment model for thin filament architecture in skeletal muscle.Nature reviews. Molecular cell biology · 2013Article
- Development transitions of thin filament proteins in rat extraocular muscles.Experimental cell research · 2013Article
- The sarcomeric protein nebulin: another multifunctional giant in charge of muscle strength optimization.Frontiers in physiology · 2012Article
- Hyperphosphorylation of mouse cardiac titin contributes to transverse aortic constriction-induced diastolic dysfunction.Circulation research · 2011Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 3 countries.
Funding
Abstract
We studied differential splicing of nebulin, a giant filamentous F-actin binding protein (M(r) approximately 700-800kDa) that is found in skeletal muscle. Nebulin spans the thin filament length, its C-terminus is anchored in the Z-disc, and its N-terminal region is located toward the thin filament pointed end. Various lines of evidence indicate that nebulin plays important roles in thin filament and Z-disc structure in skeletal muscle. In the present work we studied nebulin in a range of muscle types during postnatal development and performed transcript studies with a mouse nebulin exon microarray, developed by us, whose results were confirmed by RT-PCR. We also performed protein studies with high-resolution SDS-agarose gels and Western blots, and structural studies with electron microscopy. We found during postnatal development of the soleus muscle major changes in splicing in both the super-repeat region and the Z-disc region of nebulin; interestingly, these changes were absent in other muscle types. Three novel Z-disc exons, previously described in the mouse gene, were upregulated during postnatal development of soleus muscle and this was correlated with a significant increase in Z-disc width. These findings support the view that nebulin plays an important role in Z-disc width regulation. In summary, we discovered changes in both the super-repeat region and the Z-disc region of nebulin, that these changes are muscle-type specific, and that they correlate with differences in sarcomere structure.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.