ArticleMolecular biology of the cell2016
Nebulette is a powerful cytolinker organizing desmin and actin in mouse hearts.
Article in Molecular biology of the cell, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 38 citations in OpenAlex.
- Too rigid to strike: Excess desmin at Z-discs underlies a restrictive cardiomyopathy in GAN mice.iScience · 2026Article
- Novel truncating Desmin mutation Arg150Stop disrupts structural integrity and cellular homeostasis by formation of persistent aggregate-like structure.Cell death discovery · 2026Article
- The desmoglein 2 interactome in primary neonatal cardiomyocytes.Journal of cell science · 2026Article
- Combined Adaptive Immune Mechanisms Mediate Cardiac Injury After COVID-19 Vaccination.Circulation · 2025Article
- Unveiling the Spectrum of Minor Genes in Cardiomyopathies: A Narrative Review.International journal of molecular sciences · 2024Review
- Nucleus Mechanosensing in Cardiomyocytes.International journal of molecular sciences · 2023Review
- RNAseq profiling of blood from patients with coronary artery disease: Signature of a T cell imbalance.Journal of molecular and cellular cardiology plus · 2023Article
- Myofilament-associated proteins with intrinsic disorder (MAPIDs) and their resolution by computational modeling.Quarterly reviews of biophysics · 2023Review
- Outlining cardiac ion channel protein interactors and their signature in the human electrocardiogram.Nature cardiovascular research · 2023Article
- Sarcomere maturation: function acquisition, molecular mechanism, and interplay with other organelles.Philosophical transactions of the Royal Society of London. Series B, Biological sciences · 2022Review
- Whole exome sequencing in Brugada and long QT syndromes revealed novel rare and potential pathogenic mutations related to the dysfunction of the cardiac sodium channel.Orphanet journal of rare diseases · 2022Article
- What does desmin do: A bibliometric assessment of the functions of the muscle intermediate filament.Experimental biology and medicine (Maywood, N.J.) · 2022Article
- Understanding the molecular basis of cardiomyopathy.American journal of physiology. Heart and circulatory physiology · 2022Review
- APPRIS: selecting functionally important isoforms.Nucleic acids research · 2022Article
- Article
- Novel insights into sarcomere regulatory systems control of cardiac thin filament activation.The Journal of general physiology · 2021Article
- Deficiency in nebulin repeats of sarcomeric nebulette is detrimental for cardiomyocyte tolerance to exercise and biomechanical stress.American journal of physiology. Heart and circulatory physiology · 2021Article
- Absence of Desmin Results in Impaired Adaptive Response to Mechanical Overloading of Skeletal Muscle.Frontiers in cell and developmental biology · 2021Article
- An analysis of tissue-specific alternative splicing at the protein level.PLoS computational biology · 2020Article
- Actin Mutations and Their Role in Disease.International journal of molecular sciences · 2020Review
Corrections and comments
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In the hearts of patients bearing nebulette mutations, a severe general disorganization in cardiomyocytes of the extrasarcomeric desmin intermediate filament system is frequently observed. However, the molecular and functional relationship between the desmin cytoskeleton and nebulette-containing sarcomeres is still unclear. Here we report a high-affinity in vitro interaction between nebulette and desmin filaments. A major interaction site has been mapped to the desmin α-helical rod domain, indicating that the filament core is directly involved in the binding of nebulette. The disease-mutant desmin variants E245D and T453I exhibited increased binding affinity for nebulette, delayed filament assembly kinetics, and caused significant weakening of networks. In isolated chick cardiomyocytes and sections from canine heart, we revealed by ground-state depletion and confocal microscopies that module 5 of nebulette extends outward from Z-disk-associated desmin filaments toward the center of the sarcomere. Accordingly, in the myocardium of Des
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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.