Primary Cilia and Their Role in Acquired Heart Disease
Primary cilia are non-motile plasma membrane extrusions that display a variety of receptors and mechanosensors. Loss of function results in ciliopathies, which have been strongly linked with congenital heart disease, as well as abnormal development and function of most organ systems. Adults with con...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-03-01
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| Series: | Cells |
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| Online Access: | https://www.mdpi.com/2073-4409/11/6/960 |
| _version_ | 1797472405210267648 |
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| author | Zachariah E. Hale Junichi Sadoshima |
| author_facet | Zachariah E. Hale Junichi Sadoshima |
| author_sort | Zachariah E. Hale |
| collection | DOAJ |
| description | Primary cilia are non-motile plasma membrane extrusions that display a variety of receptors and mechanosensors. Loss of function results in ciliopathies, which have been strongly linked with congenital heart disease, as well as abnormal development and function of most organ systems. Adults with congenital heart disease have high rates of acquired heart failure, and usually die from a cardiac cause. Here we explore primary cilia’s role in acquired heart disease. Intraflagellar Transport 88 knockout results in reduced primary cilia, and knockout from cardiac endothelium produces myxomatous degeneration similar to mitral valve prolapse seen in adult humans. Induced primary cilia inactivation by other mechanisms also produces excess myocardial hypertrophy and altered scar architecture after ischemic injury, as well as hypertension due to a lack of vascular endothelial nitric oxide synthase activation and the resultant left ventricular dysfunction. Finally, primary cilia have cell-to-cell transmission capacity which, when blocked, leads to progressive left ventricular hypertrophy and heart failure, though this mechanism has not been fully established. Further research is still needed to understand primary cilia’s role in adult cardiac pathology, especially heart failure. |
| first_indexed | 2024-03-09T20:01:18Z |
| format | Article |
| id | doaj.art-c4a7eafe7cd248f69cfdb5c4f1e3cf18 |
| institution | Directory Open Access Journal |
| issn | 2073-4409 |
| language | English |
| last_indexed | 2024-03-09T20:01:18Z |
| publishDate | 2022-03-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cells |
| spelling | doaj.art-c4a7eafe7cd248f69cfdb5c4f1e3cf182023-11-24T00:43:53ZengMDPI AGCells2073-44092022-03-0111696010.3390/cells11060960Primary Cilia and Their Role in Acquired Heart DiseaseZachariah E. Hale0Junichi Sadoshima1Department of Internal Medicine-Pediatrics, Rutgers New Jersey Medical School, Newark, NJ 07103, USADepartment of Cell Biology and Molecular Medicine, Cardiovascular Research Institute, Rutgers New Jersey Medical School, Newark, NJ 07103, USAPrimary cilia are non-motile plasma membrane extrusions that display a variety of receptors and mechanosensors. Loss of function results in ciliopathies, which have been strongly linked with congenital heart disease, as well as abnormal development and function of most organ systems. Adults with congenital heart disease have high rates of acquired heart failure, and usually die from a cardiac cause. Here we explore primary cilia’s role in acquired heart disease. Intraflagellar Transport 88 knockout results in reduced primary cilia, and knockout from cardiac endothelium produces myxomatous degeneration similar to mitral valve prolapse seen in adult humans. Induced primary cilia inactivation by other mechanisms also produces excess myocardial hypertrophy and altered scar architecture after ischemic injury, as well as hypertension due to a lack of vascular endothelial nitric oxide synthase activation and the resultant left ventricular dysfunction. Finally, primary cilia have cell-to-cell transmission capacity which, when blocked, leads to progressive left ventricular hypertrophy and heart failure, though this mechanism has not been fully established. Further research is still needed to understand primary cilia’s role in adult cardiac pathology, especially heart failure.https://www.mdpi.com/2073-4409/11/6/960primary ciliacongenital heart diseaseciliopathycardiomyopathyheart failure |
| spellingShingle | Zachariah E. Hale Junichi Sadoshima Primary Cilia and Their Role in Acquired Heart Disease Cells primary cilia congenital heart disease ciliopathy cardiomyopathy heart failure |
| title | Primary Cilia and Their Role in Acquired Heart Disease |
| title_full | Primary Cilia and Their Role in Acquired Heart Disease |
| title_fullStr | Primary Cilia and Their Role in Acquired Heart Disease |
| title_full_unstemmed | Primary Cilia and Their Role in Acquired Heart Disease |
| title_short | Primary Cilia and Their Role in Acquired Heart Disease |
| title_sort | primary cilia and their role in acquired heart disease |
| topic | primary cilia congenital heart disease ciliopathy cardiomyopathy heart failure |
| url | https://www.mdpi.com/2073-4409/11/6/960 |
| work_keys_str_mv | AT zachariahehale primaryciliaandtheirroleinacquiredheartdisease AT junichisadoshima primaryciliaandtheirroleinacquiredheartdisease |