Ion channels as biomarkers of altered myogenesis in myofiber precursors of Duchenne muscular dystrophy Article Swipe
Alessandro Giovanni Cerchiara
,
Paola Imbrici
,
Raffaella Quarta
,
Enrica Cristiano
,
Brigida Boccanegra
,
Erika Caputo
,
Dominic J. Wells
,
Ornella Cappellari
,
Annamaria De Luca
·
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1111/nyas.15124
YOU?
·
· 2024
· Open Access
·
· DOI: https://doi.org/10.1111/nyas.15124
Myogenesis is essential for skeletal muscle formation, growth, and regeneration and can be altered in Duchenne muscular dystrophy (DMD), an X‐linked disorder due to the absence of the cytoskeletal protein dystrophin. Ion channels play a pivotal role in muscle differentiation and interact with the dystrophin complex. To investigate ion channel involvement in myogenesis in dystrophic settings, we performed electrophysiological characterization of two immortalized mouse cell lines, wild‐type (WT) H2K‐2B4 and the dystrophic (DYS) H2K‐SF1, and measured gene expression of differentiation markers and ion channels. Inward and outward currents/density increased as differentiation progressed in both WT and DYS cells. However, day‐11 DYS cells showed higher (27%) inward current density with an increased expression ratio of Scn5a / Scn4a and decreased (48%) barium‐sensitive outward current compared to WT. Furthermore, day‐11 DYS cells showed more positive resting membrane potential (+10 mV) and lower membrane capacitance (50%) compared to WT. DYS cells also had reduced Myog and Myf5 expression at days 6 and 11. Overall, ion channel profile and myogenesis appeared altered in DYS cells. These results are a first step in validating ion channels as potential drug targets to ameliorate muscle degeneration in DMD settings and as differentiation biomarkers in innovative platforms.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1111/nyas.15124
- OA Status
- green
- Cited By
- 2
- References
- 86
- Related Works
- 10
- OpenAlex ID
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All OpenAlex metadata
Raw OpenAlex JSON
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https://openalex.org/W4393067127Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1111/nyas.15124Digital Object Identifier
- Title
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Ion channels as biomarkers of altered myogenesis in myofiber precursors of Duchenne muscular dystrophyWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
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2024Year of publication
- Publication date
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2024-03-22Full publication date if available
- Authors
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Alessandro Giovanni Cerchiara, Paola Imbrici, Raffaella Quarta, Enrica Cristiano, Brigida Boccanegra, Erika Caputo, Dominic J. Wells, Ornella Cappellari, Annamaria De LucaList of authors in order
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https://doi.org/10.1111/nyas.15124Publisher landing page
- Open access
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YesWhether a free full text is available
- OA status
-
greenOpen access status per OpenAlex
- OA URL
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https://hdl.handle.net/11586/469220Direct OA link when available
- Concepts
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Myogenesis, Duchenne muscular dystrophy, Myocyte, Chemistry, Muscular dystrophy, Cell biology, Internal medicine, Medicine, BiologyTop concepts (fields/topics) attached by OpenAlex
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2Total citation count in OpenAlex
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2025: 2Per-year citation counts (last 5 years)
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86Number of works referenced by this work
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10Other works algorithmically related by OpenAlex
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