The DOI matches its Crossref record; 24 of the 24 references it cites were found in a registry or an index.
Expression of R120G–αB-Crystallin Causes Aberrant Desmin and αB-Crystallin Aggregation and Cardiomyopathy in Mice
Open accessAbstract
Abstract —Upregulation of αB-crystallin (CryAB), a small heat shock protein, is associated with a variety of diseases, including the desmin-related myopathies. CryAB, which binds to both desmin and cytoplasmic actin, may participate as a chaperone in intermediate filament formation and maintenance, but the physiological consequences of CryAB upregulation are unknown. A mutation in CryAB, R120G, has been linked to a familial desminopathy. However, it is unclear whether the mutation is directly causative. We created multiple transgenic mouse lines that overexpressed either murine wild-type CryAB or the R120G mutation in cardiomyocytes. Overexpression of wild-type CryAB was relatively benign, with no increases in mortality and no induction of desmin-related cardiomyopathy even in a line in which CryAB mRNA expression was increased ≈104-fold and the protein level increased by 11-fold. In contrast, lines expressing the R120G mutation were compromised, with a high-expressing line exhibiting 100% mortality by early adulthood. Modest expression levels resulted in a phenotype that was strikingly similar to that observed for the desmin-related cardiomyopathies. The desmin filaments in the cardiomyocytes were overtly affected, myofibril alignment was significantly impaired, and a hypertrophic response occurred at both the molecular and cellular levels. The data show that the R120G mutation causes a desminopathy, is dominant negative, and results in cardiac hypertrophy.
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Cites
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Enhanced autophagy ameliorates cardiac proteinopathy
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Self-citationProteostasis in cardiac health and disease
Henning, R. H., & Brundel, B. J. J. M. (2017) · Nature Reviews Cardiology · doi:10.1038/nrcardio.2017.89 · Cited by 193
Autophagy and p62 in Cardiac Proteinopathy
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Metadata
- Authors
- Wang, X., Osinska, H., Klevitsky, R., Gerdes, A. M., Nieman, M., Lorenz, J., Hewett, T., & Robbins, J.
- Venue
- Circulation Research
- Published
- 2001-07-06
- Volume
- 89
- Issue
- 1
- Pages
- 84-91
- ISSN
- 0009-7330 (ISSN-L)
- 1524-4571
- OpenAlex
- W2127812911
- Cited by
- 309
- References
- 24
- FWCI
- 2.82
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Wang, X., Osinska, H., Klevitsky, R., Gerdes, A. M., Nieman, M., Lorenz, J., Hewett, T., & Robbins, J. (2001). Expression of R120G–αB-Crystallin Causes Aberrant Desmin and αB-Crystallin Aggregation and Cardiomyopathy in Mice. Circulation Research. https://doi.org/10.1161/hh1301.092688
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