Ankrd2 in Mechanotransduction and Oxidative Stress Response in Skeletal Muscle: New Cues for the Pathogenesis of Muscular Laminopathies
| Autor: | Giovanna Lattanzi, Vittoria Cenni, Snezana Kojic, Cristina Capanni, Georgine Faulkner |
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| Jazyk: | angličtina |
| Rok vydání: | 2019 |
| Předmět: |
ANKRD2
Aging congenital hereditary and neonatal diseases and abnormalities Muscle Proteins Review Article Biology Biochemistry Mechanotransduction Cellular 03 medical and health sciences PEST sequence 0302 clinical medicine medicine Myocyte Humans Protein Isoforms Muscular dystrophy Mechanotransduction lcsh:QH573-671 Muscle Skeletal 030304 developmental biology 0303 health sciences lcsh:Cytology Myocardium Skeletal muscle Nuclear Proteins Cell Biology General Medicine medicine.disease Lamin Type A Muscular Dystrophy Emery-Dreifuss Cell biology Repressor Proteins Oxidative Stress medicine.anatomical_structure Ankyrin repeat Reactive Oxygen Species 030217 neurology & neurosurgery Lamin |
| Zdroj: | Oxidative Medicine and Cellular Longevity, Vol 2019 (2019) Oxidative Medicine and Cellular Longevity |
| ISSN: | 1942-0994 1942-0900 |
| Popis: | Ankrd2 (ankyrin repeats containing domain 2) or Arpp (ankyrin repeat, PEST sequence, and proline-rich region) is a member of the muscle ankyrin repeat protein family. Ankrd2 is mostly expressed in skeletal muscle, where it plays an intriguing role in the transcriptional response to stress induced by mechanical stimulation as well as by cellular reactive oxygen species. Our studies in myoblasts from Emery-Dreifuss muscular dystrophy 2, a LMNA-linked disease affecting skeletal and cardiac muscles, demonstrated that Ankrd2 is a lamin A-binding protein and that mutated lamins found in Emery-Dreifuss muscular dystrophy change the dynamics of Ankrd2 nuclear import, thus affecting oxidative stress response. In this review, besides describing the latest advances related to Ankrd2 studies, including novel discoveries on Ankrd2 isoform-specific functions, we report the main findings on the relationship of Ankrd2 with A-type lamins and discuss known and potential mechanisms involving defective Ankrd2-lamin A interplay in the pathogenesis of muscular laminopathies. |
| Databáze: | OpenAIRE |
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