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Loss of Enigma Homolog Protein Results in Dilated Cardiomyopathy
Open accessAbstract
Rationale: The Z-line, alternatively termed the Z-band or Z-disc, is a highly ordered structure at the border between 2 sarcomeres. Enigma subfamily proteins (Enigma, Enigma homolog protein, and Cypher) of the PDZ-LIM domain protein family are Z-line proteins. Among the Enigma subfamily, Cypher has been demonstrated to play a pivotal role in the structure and function of striated muscle, whereas the role of Enigma homolog protein (ENH) in muscle remains largely unknown. Objective: We studied the role of Enigma homolog protein in the heart using global and cardiac-specific ENH knockout mouse models. Methods and Results: We identified new exons and splice isoforms for ENH in the mouse heart. Impaired cardiac contraction and dilated cardiomyopathy were observed in ENH null mice. Mice with cardiac specific ENH deletion developed a similar dilated cardiomyopathy. Like Cypher, ENH interacted with Calsarcin-1, another Z-line protein. Moreover, biochemical studies showed that ENH, Cypher short isoform and Calsarcin-1 are within the same protein complex at the Z-line. Cypher short isoform and Calsarcin-1 proteins are specifically downregulated in ENH null hearts. Conclusions: We have identified an ENH-CypherS-Calsarcin protein complex at the Z-line. Ablation of ENH leads to destabilization of this protein complex and dilated cardiomyopathy.
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“ENH is a family of PDZ-LIM domain proteins that localize to Z-disk and serve as scaffolds for signaling complexes (65).”
From Distinct hypertrophic cardiomyopathy genotypes result in convergent sarcomeric proteoform profiles revealed by top-down proteomics (2020) · cites this paper as [65]
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Background
Metadata
- Authors
- Cheng, H., Kimura, K., Peter, A. K., Cui, L., Ouyang, K., Shen, T., Liu, Y., Gu, Y., Dalton, N. D., Evans, S. M., Knowlton, K. U., Peterson, K. L., & Chen, J.
- Affiliations
- University of California San Diego (US)
- From the Department of Medicine, University of California–San Diego, La Jolla, Calif.
- ORCID
- Venue
- Circulation Research
- Published
- 2010-08-06
- Volume
- 107
- Issue
- 3
- Pages
- 348-356
- ISSN
- 0009-7330 (ISSN-L)
- 1524-4571
- PMC
- PMC3684396
- OpenAlex
- W2004101138
- Cited by
- 106
- References
- 45
- Citations by year
- 2026: 4 · 2025: 4 · 2024: 6 · 2023: 5 · 2022: 8 · 2021: 6 · 2020: 8 · 2019: 6 · 2018: 1 · 2017: 6 · 2016: 9 · 2015: 9 · 2014: 6 · 2013: 8 · 2012: 11
- FWCI
- 6.15
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Circulation Research (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health (1r01hl106968-01)
- National Heart, Lung, and Blood Institute (R01-HL106968, P01HL046345, R01HL066100, T32HL007444)
- Topics
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiomyopathy and Myosin Studies
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac electrophysiology and arrhythmias
- Keywords
- dilated cardiomyopathy, Z-line, Cypher, sarcomere, cardiac contraction, protein complexes, cardiac-specific knockout mice, striated muscle
- MeSH terms
- Animals, Cardiomyopathy, Dilated, Carrier Proteins, Exons, Microfilament Proteins, Muscle Proteins, Myocardial Contraction, Plasmids, Systole, Polymerase Chain Reaction, Alternative Splicing, Mice, Knockout, Homeodomain Proteins, Protein Isoforms, Adaptor Proteins, Signal Transducing, Mice, LIM Domain Proteins
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