The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice
Abstract Fibrillin‐1 mutations cause pathological changes in connective tissue that constitute the complex phenotype of Marfan syndrome. In this study, we used fibrillin‐1 hypomorphic and haploinsufficient mice (Fbn1mgr/mgR and Fbn1+/− mice, respectively) to investigate the impact of fibrillin‐1 def...
| Main Authors: | , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2019-11-01
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| Series: | Physiological Reports |
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| Online Access: | https://doi.org/10.14814/phy2.14267 |
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| author | Peter H. T. Tran Tanja Skrba Elisabeth Wondimu Giuseppina Galatioto René Brüggebusch Svensson Annesofie T. Olesen Abigail L. Mackey S. Peter Magnusson Francesco Ramirez Michael Kjaer |
| author_facet | Peter H. T. Tran Tanja Skrba Elisabeth Wondimu Giuseppina Galatioto René Brüggebusch Svensson Annesofie T. Olesen Abigail L. Mackey S. Peter Magnusson Francesco Ramirez Michael Kjaer |
| author_sort | Peter H. T. Tran |
| collection | DOAJ |
| description | Abstract Fibrillin‐1 mutations cause pathological changes in connective tissue that constitute the complex phenotype of Marfan syndrome. In this study, we used fibrillin‐1 hypomorphic and haploinsufficient mice (Fbn1mgr/mgR and Fbn1+/− mice, respectively) to investigate the impact of fibrillin‐1 deficiency alone or in combination with regular physical activity on tendon tissue morphology and mechanical properties. Morphological and biomechanical analyses revealed that Fbn1mgr/mgR but not Fbn1+/− mice displayed smaller tendons with physical properties that were unremarkable when normalized to tendon size. Fbn1mgR/mgR mice (n = 43) Fbn1+/−mice (n = 27) and wild‐type mice (WT, n = 25) were randomly assigned to either control cage conditions (n = 54) or to a running on a running wheel for 4 weeks (n = 41). Both fibrillin‐1‐deficient mice ran voluntarily on the running wheel in a manner similar to WT mice (3–4 km/24 h). Regular exercise did not mitigate aneurysm progression in Fbn1mgR/mgR mice (P < 0.05) as evidenced by unmodified median survival. In spite of the smaller size, tendons of fibrillin‐1‐deficient mice subjected to regular exercise showed no evidence of overt histopathological changes or tissue overload. We therefore concluded that lack of optimal fibrillin‐1 synthesis leads to a down regulation of integrated tendon formation, rather than to a loss of tendon quality, which also implies that fibrillin‐1 deficiency in combination with exercise is not a suitable animal model for studying the development of tendon overuse (tendinopathy). |
| first_indexed | 2024-04-13T17:46:53Z |
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| id | doaj.art-5aa38c661acc4c35a8cc0a238faa86e1 |
| institution | Directory Open Access Journal |
| issn | 2051-817X |
| language | English |
| last_indexed | 2024-04-13T17:46:53Z |
| publishDate | 2019-11-01 |
| publisher | Wiley |
| record_format | Article |
| series | Physiological Reports |
| spelling | doaj.art-5aa38c661acc4c35a8cc0a238faa86e12022-12-22T02:36:56ZengWileyPhysiological Reports2051-817X2019-11-01721n/an/a10.14814/phy2.14267The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in micePeter H. T. Tran0Tanja Skrba1Elisabeth Wondimu2Giuseppina Galatioto3René Brüggebusch Svensson4Annesofie T. Olesen5Abigail L. Mackey6S. Peter Magnusson7Francesco Ramirez8Michael Kjaer9Department of Pharmacological Sciences Icahn School of Medicine at Mount Sinai New York New YorkDepartment of Pharmacological Sciences Icahn School of Medicine at Mount Sinai New York New YorkDepartment of Pharmacological Sciences Icahn School of Medicine at Mount Sinai New York New YorkDepartment of Pharmacological Sciences Icahn School of Medicine at Mount Sinai New York New YorkInstitute of Sports Medicine Department of Orthopedic Surgery M Bispebjerg Hospital Copenhagen DenmarkInstitute of Sports Medicine Department of Orthopedic Surgery M Bispebjerg Hospital Copenhagen DenmarkInstitute of Sports Medicine Department of Orthopedic Surgery M Bispebjerg Hospital Copenhagen DenmarkDepartment of Physical and Occupational Therapy Bispebjerg Hospital Copenhagen DenmarkDepartment of Pharmacological Sciences Icahn School of Medicine at Mount Sinai New York New YorkInstitute of Sports Medicine Department of Orthopedic Surgery M Bispebjerg Hospital Copenhagen DenmarkAbstract Fibrillin‐1 mutations cause pathological changes in connective tissue that constitute the complex phenotype of Marfan syndrome. In this study, we used fibrillin‐1 hypomorphic and haploinsufficient mice (Fbn1mgr/mgR and Fbn1+/− mice, respectively) to investigate the impact of fibrillin‐1 deficiency alone or in combination with regular physical activity on tendon tissue morphology and mechanical properties. Morphological and biomechanical analyses revealed that Fbn1mgr/mgR but not Fbn1+/− mice displayed smaller tendons with physical properties that were unremarkable when normalized to tendon size. Fbn1mgR/mgR mice (n = 43) Fbn1+/−mice (n = 27) and wild‐type mice (WT, n = 25) were randomly assigned to either control cage conditions (n = 54) or to a running on a running wheel for 4 weeks (n = 41). Both fibrillin‐1‐deficient mice ran voluntarily on the running wheel in a manner similar to WT mice (3–4 km/24 h). Regular exercise did not mitigate aneurysm progression in Fbn1mgR/mgR mice (P < 0.05) as evidenced by unmodified median survival. In spite of the smaller size, tendons of fibrillin‐1‐deficient mice subjected to regular exercise showed no evidence of overt histopathological changes or tissue overload. We therefore concluded that lack of optimal fibrillin‐1 synthesis leads to a down regulation of integrated tendon formation, rather than to a loss of tendon quality, which also implies that fibrillin‐1 deficiency in combination with exercise is not a suitable animal model for studying the development of tendon overuse (tendinopathy).https://doi.org/10.14814/phy2.14267TendinopathyExerciseMarfanSurvivalBiomechanics |
| spellingShingle | Peter H. T. Tran Tanja Skrba Elisabeth Wondimu Giuseppina Galatioto René Brüggebusch Svensson Annesofie T. Olesen Abigail L. Mackey S. Peter Magnusson Francesco Ramirez Michael Kjaer The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice Physiological Reports Tendinopathy Exercise Marfan Survival Biomechanics |
| title | The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| title_full | The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| title_fullStr | The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| title_full_unstemmed | The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| title_short | The influence of fibrillin‐1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| title_sort | influence of fibrillin 1 and physical activity upon tendon tissue morphology and mechanical properties in mice |
| topic | Tendinopathy Exercise Marfan Survival Biomechanics |
| url | https://doi.org/10.14814/phy2.14267 |
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