ArticleDisease models & mechanisms2015
Leiomodin-3-deficient mice display nemaline myopathy with fast-myofiber atrophy.
Article in Disease models & mechanisms, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 33 citations in OpenAlex.
- Proteomic Analysis of Thin Filament Components Elucidates Changes in Spastic Muscle Sarcomere After Stroke.International journal of molecular sciences · 2025Article
- A nemaline myopathy-linked mutation inhibits the actin-regulatory functions of tropomodulin and leiomodin.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- Article
- p300 and cAMP response element-binding protein-binding protein in skeletal muscle homeostasis, contractile function, and survival.Journal of cachexia, sarcopenia and muscle · 2020Article
- Article
- Nemaline myopathies: a current view.Journal of muscle research and cell motility · 2019Review
- Functional regulatory mechanism of smooth muscle cell-restricted LMOD1 coronary artery disease locus.PLoS genetics · 2018Article
- Neonatal fractures as a presenting feature of LMOD3-associated congenital myopathy.American journal of medical genetics. Part A · 2017Article
- Overview of the Muscle Cytoskeleton.Comprehensive Physiology · 2017Review
- Tropomodulins and Leiomodins: Actin Pointed End Caps and Nucleators in Muscles.Biophysical journal · 2017Review
- Loss of LMOD1 impairs smooth muscle cytocontractility and causes megacystis microcolon intestinal hypoperistalsis syndrome in humans and mice.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- Exome sequencing reveals a nebulin nonsense mutation in a dog model of nemaline myopathy.Mammalian genome : official journal of the International Mammalian Genome Society · 2016Article
- Nebulin, a multi-functional giant.The Journal of experimental biology · 2016Review
- Lmod2 piggyBac mutant mice exhibit dilated cardiomyopathy.Cell & bioscience · 2016Article
- Tropomodulin 1 directly controls thin filament length in both wild-type and tropomodulin 4-deficient skeletal muscle.Development (Cambridge, England) · 2015Article
- LMOD3: the "missing link" in nemaline myopathy?Oncotarget · 2015Article
- Sarcomere Dysfunction in Nemaline Myopathy.Journal of neuromuscular diseasesReview
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Authors and funding
8 authors at 4 institutions in 2 countries.
Funding
Abstract
Nemaline myopathy (NM) is one of the most common forms of congenital myopathy, and affects either fast myofibers, slow myofibers, or both. However, an animal model for congenital myopathy with fast-myofiber-specific atrophy is not available. Furthermore, mutations in the leiomodin-3 (LMOD3) gene have recently been identified in a group of individuals with NM. However, it is not clear how loss of LMOD3 leads to NM. Here, we report a mouse mutant in which the piggyBac (PB) transposon is inserted into the Lmod3 gene and disrupts its expression. Lmod3(PB/PB) mice show severe muscle weakness and postnatal growth retardation. Electron microscopy and immunofluorescence studies of the mutant skeletal muscles revealed the presence of nemaline bodies, a hallmark of NM, and disorganized sarcomeric structures. Interestingly, Lmod3 deficiency caused muscle atrophy specific to the fast fibers. Together, our results show that Lmod3 is required in the fast fibers for sarcomere integrity, and this study offers the first NM mouse model with muscle atrophy that is specific to fast fibers. This model could be a valuable resource for interrogating myopathy pathogenesis and developing therapeutics for NM as well as other pathophysiological conditions with preferential atrophy of fast fibers, including cancer cachexia and sarcopenia.
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