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Nuclear Small Dystrophin Isoforms during Muscle Differentiation

Mutations in the DMD gene can cause Duchenne or Becker muscular dystrophy (DMD/BMD) by affecting the giant isoform of dystrophin, a protein encoded by the DMD gene. The role of small dystrophin isoforms is not well investigated yet, and they may play a role in muscle development and molecular pathol...

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Autores principales: Donandt, Tina, Todorow, Vanessa, Hintze, Stefan, Graupner, Alexandra, Schoser, Benedikt, Walter, Maggie C., Meinke, Peter
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10302385/
https://www.ncbi.nlm.nih.gov/pubmed/37374149
http://dx.doi.org/10.3390/life13061367
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author Donandt, Tina
Todorow, Vanessa
Hintze, Stefan
Graupner, Alexandra
Schoser, Benedikt
Walter, Maggie C.
Meinke, Peter
author_facet Donandt, Tina
Todorow, Vanessa
Hintze, Stefan
Graupner, Alexandra
Schoser, Benedikt
Walter, Maggie C.
Meinke, Peter
author_sort Donandt, Tina
collection PubMed
description Mutations in the DMD gene can cause Duchenne or Becker muscular dystrophy (DMD/BMD) by affecting the giant isoform of dystrophin, a protein encoded by the DMD gene. The role of small dystrophin isoforms is not well investigated yet, and they may play a role in muscle development and molecular pathology. Here, we investigated the nuclear localization of short carboxy-terminal dystrophin isoforms during the in vitro differentiation of human, porcine, and murine myoblast cultures. We could not only confirm the presence of Dp71 in the nucleoplasm and at the nuclear envelope, but we could also identify the Dp40 isoform in muscle nuclei. The localization of both isoforms over the first six days of differentiation was similar between human and porcine myoblasts, but murine myoblasts behaved differently. This highlights the importance of the porcine model in investigating DMD. We could also detect a wave-like pattern of nuclear presence of both Dp71 and Dp40, indicating a direct or indirect involvement in gene expression control during muscle differentiation.
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spelling pubmed-103023852023-06-29 Nuclear Small Dystrophin Isoforms during Muscle Differentiation Donandt, Tina Todorow, Vanessa Hintze, Stefan Graupner, Alexandra Schoser, Benedikt Walter, Maggie C. Meinke, Peter Life (Basel) Brief Report Mutations in the DMD gene can cause Duchenne or Becker muscular dystrophy (DMD/BMD) by affecting the giant isoform of dystrophin, a protein encoded by the DMD gene. The role of small dystrophin isoforms is not well investigated yet, and they may play a role in muscle development and molecular pathology. Here, we investigated the nuclear localization of short carboxy-terminal dystrophin isoforms during the in vitro differentiation of human, porcine, and murine myoblast cultures. We could not only confirm the presence of Dp71 in the nucleoplasm and at the nuclear envelope, but we could also identify the Dp40 isoform in muscle nuclei. The localization of both isoforms over the first six days of differentiation was similar between human and porcine myoblasts, but murine myoblasts behaved differently. This highlights the importance of the porcine model in investigating DMD. We could also detect a wave-like pattern of nuclear presence of both Dp71 and Dp40, indicating a direct or indirect involvement in gene expression control during muscle differentiation. MDPI 2023-06-11 /pmc/articles/PMC10302385/ /pubmed/37374149 http://dx.doi.org/10.3390/life13061367 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Brief Report
Donandt, Tina
Todorow, Vanessa
Hintze, Stefan
Graupner, Alexandra
Schoser, Benedikt
Walter, Maggie C.
Meinke, Peter
Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title_full Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title_fullStr Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title_full_unstemmed Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title_short Nuclear Small Dystrophin Isoforms during Muscle Differentiation
title_sort nuclear small dystrophin isoforms during muscle differentiation
topic Brief Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10302385/
https://www.ncbi.nlm.nih.gov/pubmed/37374149
http://dx.doi.org/10.3390/life13061367
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