Dysferlin Regulates Cell Adhesion in Human Monocytes
| العنوان: | Dysferlin Regulates Cell Adhesion in Human Monocytes |
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| المؤلفون: | Laure Grand Moursel, Rune R. Frants, Jun Wang, René E. M. Toes, Ivana Bagaric, Silvère M. van der Maarel, Eduard Gallardo, Bàrbara Flix, Antoine de Morrée, Isabel Illa, Marlinde L. van den Boogaard |
| المصدر: | JOURNAL OF BIOLOGICAL CHEMISTRY r-IIB SANT PAU. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica Sant Pau instname Journal of Biological Chemistry, 288(20), 14147-14157 de Morrée, A, Flix, B, Bagaric, I, Wang, J, van den Boogaard, M, Grand Moursel, L, Frants, R R, Illa, I, Gallardo, E, Toes, R & van der Maarel, S M 2013, ' Dysferlin regulates cell adhesion in human monocytes ', The Journal of Biological Chemistry, vol. 288, no. 20, pp. 14147-14157 . https://doi.org/10.1074/jbc.M112.448589Test |
| بيانات النشر: | AMER SOC BIOCHEMISTRY MOLECULAR BIOLOGY INC, 2013. |
| سنة النشر: | 2013 |
| مصطلحات موضوعية: | Monocytes/cytology, Dysferlinopathy, Integrin, Cell, Muscle Proteins, Biology, Biochemistry, Monocytes, Muscular Dystrophies, Dysferlin, Cell membrane, medicine, Cell Adhesion, Humans, Muscular Dystrophies/metabolism, Membrane Proteins/genetics, Muscle, Skeletal, Cell adhesion, Molecular Biology, Inflammation, Muscle Proteins/genetics, Muscle, Skeletal/metabolism, Monocyte, Macrophages, Integrin beta3, Muscular Dystrophies, Limb-Girdle/genetics, Membrane Proteins, Cell Differentiation, Cell Biology, biochemical phenomena, metabolism, and nutrition, medicine.disease, Cell biology, Fibronectin, medicine.anatomical_structure, Muscular Dystrophies, Limb-Girdle, Gene Expression Regulation, Immune System, Mutation, biology.protein, Integrin beta3/metabolism |
| الوصف: | Dysferlin is mutated in a group of muscular dystrophies commonly referred to as dysferlinopathies. It is highly expressed in skeletal muscle, where it is important for sarcolemmal maintenance. Recent studies show that dysferlin is also expressed in monocytes. Moreover, muscle of dysferlinopathy patients is characterized by massive immune cell infiltrates, and dysferlin-negative monocytes were shown to be more aggressive and phagocytose more particles. This suggests that dysferlin deregulation in monocytes might contribute to disease progression, but the molecular mechanism is unclear. Here we show that dysferlin expression is increased with differentiation in human monocytes and the THP1 monocyte cell model. Freshly isolated monocytes of dysferlinopathy patients show deregulated expression of fibronectin and fibronectin-binding integrins, which is recapitulated by transient knockdown of dysferlin in THP1 cells. Dysferlin forms a protein complex with these integrins at the cell membrane, and its depletion impairs cell adhesion. Moreover, patient macrophages show altered adhesion and motility. These findings suggest that dysferlin is involved in regulating cellular interactions and provide new insight into dysferlin function in inflammatory cells. |
| ردمك: | 978-1-4147-1415-8 1-4147-1415-7 |
| تدمد: | 0021-9258 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ed5bf61dc43b228f7acaa8aa2ad1ec10Test https://iibsantpau.fundanetsuite.com/Publicaciones/ProdCientif/PublicacionFrw.aspx?id=10011Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....ed5bf61dc43b228f7acaa8aa2ad1ec10 |
| قاعدة البيانات: | OpenAIRE |
| ردمك: | 9781414714158 1414714157 |
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| تدمد: | 00219258 |