ArticleHuman molecular genetics2013
Defective skeletal muscle growth in lamin A/C-deficient mice is rescued by loss of Lap2α.
Article in Human molecular genetics, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers.
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Who cites it
32 citing papers in PubMed, 51 citations in OpenAlex.
- Multi-omics integration identifies molecular markers and biological pathways for carcass and meat quality traits in Nellore cattle.Scientific reports · 2025Article
- LAP2alpha facilitates myogenic gene expression by preventing nucleoplasmic lamin A/C from spreading to active chromatin regions.Nucleic acids research · 2024Article
- Pervasive nuclear envelope ruptures precede ECM signaling and disease onset without activating cGAS-STING in Lamin-cardiomyopathy mice.Cell reports · 2024Article
- Pervasive nuclear envelope ruptures precede ECM signaling and disease onset without activating cGAS-STING in Lamin-cardiomyopathy mice.bioRxiv : the preprint server for biology · 2024Article
- Zebrafish Models for Skeletal Muscle Senescence: Lessons from Cell Cultures and Rodent Models.Molecules (Basel, Switzerland) · 2022Review
- Loss of function of the nuclear envelope protein LEMD2 causes DNA damage-dependent cardiomyopathy.The Journal of clinical investigation · 2022Article
- Lamina-associated polypeptide 2α is required for intranuclear MRTF-A activity.Scientific reports · 2022Article
- Nuclear Lamins: Key Proteins for Embryonic Development.Biology · 2022Review
- Preclinical Advances of Therapies for Laminopathies.Journal of clinical medicine · 2021Review
- Disrupting the LINC complex by AAV mediated gene transduction prevents progression of Lamin induced cardiomyopathy.Nature communications · 2021Article
- Role ofBiomolecules · 2021Article
- Cardiomyocyte Proliferative Capacity Is Restricted in Mice WithFrontiers in cardiovascular medicine · 2021Article
- A Role of Lamin A/C in Preventing Neuromuscular Junction Decline in Mice.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2020Article
- Dysfunctional polycomb transcriptional repression contributes to lamin A/C-dependent muscular dystrophy.The Journal of clinical investigation · 2020Article
- The mammalian LINC complex component SUN1 regulates muscle regeneration by modulating drosha activity.eLife · 2019Article
- 2C-BioID: An Advanced Two Component BioID System for Precision Mapping of Protein Interactomes.iScience · 2018Article
- Lamins and Lamin-Associated Proteins in Gastrointestinal Health and Disease.Gastroenterology · 2018Review
- SMAD6 overexpression leads to accelerated myogenic differentiation of LMNA mutated cells.Scientific reports · 2018Article
- Nesprins and Lamins in Health and Diseases of Cardiac and Skeletal Muscles.Frontiers in physiology · 2018Review
- The Pathogenesis and Therapies of Striated Muscle Laminopathies.Frontiers in physiology · 2018Review
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 4 countries.
Funding
Abstract
Mutations in lamin A/C result in a range of tissue-specific disorders collectively called laminopathies. Of these, Emery-Dreifuss and Limb-Girdle muscular dystrophy 1B mainly affect striated muscle. A useful model for understanding both laminopathies and lamin A/C function is the Lmna(-/-) mouse. We found that skeletal muscle growth and muscle satellite (stem) cell proliferation were both reduced in Lmna(-/-) mice. Lamins A and C associate with lamina-associated polypeptide 2 alpha (Lap2α) and the retinoblastoma gene product, pRb, to regulate cell cycle exit. We found Lap2α to be upregulated in Lmna(-/-) myoblasts (MBs). To specifically test the contribution of elevated Lap2α to the phenotype of Lmna(-/-) mice, we generated Lmna(-/-)Lap2α(-/-) mice. Lifespan and body mass were increased in Lmna(-/-)Lap2α(-/-) mice compared with Lmna(-/-). Importantly, the satellite cell proliferation defect was rescued, resulting in improved myogenesis. Lmna(-/-) MBs also exhibited increased levels of Smad2/3, which were abnormally distributed in the cell and failed to respond to TGFβ1 stimulation as in control cells. However, using SIS3 to inhibit signaling via Smad3 reduced cell death and augmented MB fusion. Together, our results show that perturbed Lap2α/pRb and Smad2/3 signaling are important regulatory pathways mediating defective muscle growth in Lmna(-/-) mice, and that inhibition of either pathway alone or in combination can ameliorate this deleterious phenotype.
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