N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy
Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dyst...
Ausführliche Beschreibung
Gespeichert in:
| Autor*in: |
Falcone, Sestina [verfasserIn] Roman, William [verfasserIn] Hnia, Karim [verfasserIn] Gache, Vincent [verfasserIn] Didier, Nathalie [verfasserIn] Lainé, Jeanne [verfasserIn] Auradé, Frederic [verfasserIn] Marty, Isabelle [verfasserIn] Nishino, Ichizo [verfasserIn] Charlet‐Berguerand, Nicolas [verfasserIn] Romero, Norma Beatriz [verfasserIn] Marazzi, Giovanna [verfasserIn] Sassoon, David [verfasserIn] Laporte, Jocelyn [verfasserIn] Gomes, Edgar R [verfasserIn] |
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| Format: |
E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
2014 |
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| Anmerkung: |
© The Authors. Published under the terms of the CC BY 4.0 license 2014 |
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| Übergeordnetes Werk: |
Enthalten in: EMBO Molecular Medicine - Nature Publishing Group UK, 2023, 6(2014), 11 vom: 29. Sept., Seite 1455-1475 |
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| Übergeordnetes Werk: |
volume:6 ; year:2014 ; number:11 ; day:29 ; month:09 ; pages:1455-1475 |
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| DOI / URN: |
10.15252/emmm.201404436 |
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| Katalog-ID: |
SPR05795786X |
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| 245 | 1 | 0 | |a N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
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| 520 | |a Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. | ||
| 520 | |a Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. | ||
| 520 | |a Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. | ||
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| 700 | 1 | |a Roman, William |e verfasserin |4 aut | |
| 700 | 1 | |a Hnia, Karim |e verfasserin |4 aut | |
| 700 | 1 | |a Gache, Vincent |e verfasserin |4 aut | |
| 700 | 1 | |a Didier, Nathalie |e verfasserin |4 aut | |
| 700 | 1 | |a Lainé, Jeanne |e verfasserin |4 aut | |
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| 700 | 1 | |a Sassoon, David |e verfasserin |4 aut | |
| 700 | 1 | |a Laporte, Jocelyn |e verfasserin |4 aut | |
| 700 | 1 | |a Gomes, Edgar R |e verfasserin |4 aut | |
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10.15252/emmm.201404436 doi (DE-627)SPR05795786X (SPR)emmm.201404436-e DE-627 ger DE-627 rakwb eng Falcone, Sestina verfasserin aut N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Authors. Published under the terms of the CC BY 4.0 license 2014 Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 Roman, William verfasserin aut Hnia, Karim verfasserin aut Gache, Vincent verfasserin aut Didier, Nathalie verfasserin aut Lainé, Jeanne verfasserin aut Auradé, Frederic verfasserin aut Marty, Isabelle verfasserin aut Nishino, Ichizo verfasserin aut Charlet‐Berguerand, Nicolas verfasserin aut Romero, Norma Beatriz verfasserin aut Marazzi, Giovanna verfasserin aut Sassoon, David verfasserin aut Laporte, Jocelyn verfasserin aut Gomes, Edgar R verfasserin aut Enthalten in EMBO Molecular Medicine Nature Publishing Group UK, 2023 6(2014), 11 vom: 29. Sept., Seite 1455-1475 (DE-627)594772761 (DE-600)2485479-7 1757-4684 nnns volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 https://dx.doi.org/10.15252/emmm.201404436 X:SPRINGER Resolving-System kostenfrei Volltext SYSFLAG_0 GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_72 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4029 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4116 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4155 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4311 GBV_ILN_4313 GBV_ILN_4314 GBV_ILN_4315 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4598 GBV_ILN_4700 AR 6 2014 11 29 09 1455-1475 |
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10.15252/emmm.201404436 doi (DE-627)SPR05795786X (SPR)emmm.201404436-e DE-627 ger DE-627 rakwb eng Falcone, Sestina verfasserin aut N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Authors. Published under the terms of the CC BY 4.0 license 2014 Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 Roman, William verfasserin aut Hnia, Karim verfasserin aut Gache, Vincent verfasserin aut Didier, Nathalie verfasserin aut Lainé, Jeanne verfasserin aut Auradé, Frederic verfasserin aut Marty, Isabelle verfasserin aut Nishino, Ichizo verfasserin aut Charlet‐Berguerand, Nicolas verfasserin aut Romero, Norma Beatriz verfasserin aut Marazzi, Giovanna verfasserin aut Sassoon, David verfasserin aut Laporte, Jocelyn verfasserin aut Gomes, Edgar R verfasserin aut Enthalten in EMBO Molecular Medicine Nature Publishing Group UK, 2023 6(2014), 11 vom: 29. Sept., Seite 1455-1475 (DE-627)594772761 (DE-600)2485479-7 1757-4684 nnns volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 https://dx.doi.org/10.15252/emmm.201404436 X:SPRINGER Resolving-System kostenfrei Volltext SYSFLAG_0 GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_72 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4029 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4116 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4155 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4311 GBV_ILN_4313 GBV_ILN_4314 GBV_ILN_4315 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4598 GBV_ILN_4700 AR 6 2014 11 29 09 1455-1475 |
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10.15252/emmm.201404436 doi (DE-627)SPR05795786X (SPR)emmm.201404436-e DE-627 ger DE-627 rakwb eng Falcone, Sestina verfasserin aut N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Authors. Published under the terms of the CC BY 4.0 license 2014 Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 Roman, William verfasserin aut Hnia, Karim verfasserin aut Gache, Vincent verfasserin aut Didier, Nathalie verfasserin aut Lainé, Jeanne verfasserin aut Auradé, Frederic verfasserin aut Marty, Isabelle verfasserin aut Nishino, Ichizo verfasserin aut Charlet‐Berguerand, Nicolas verfasserin aut Romero, Norma Beatriz verfasserin aut Marazzi, Giovanna verfasserin aut Sassoon, David verfasserin aut Laporte, Jocelyn verfasserin aut Gomes, Edgar R verfasserin aut Enthalten in EMBO Molecular Medicine Nature Publishing Group UK, 2023 6(2014), 11 vom: 29. Sept., Seite 1455-1475 (DE-627)594772761 (DE-600)2485479-7 1757-4684 nnns volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 https://dx.doi.org/10.15252/emmm.201404436 X:SPRINGER Resolving-System kostenfrei Volltext SYSFLAG_0 GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_72 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4029 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4116 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4155 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4311 GBV_ILN_4313 GBV_ILN_4314 GBV_ILN_4315 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4598 GBV_ILN_4700 AR 6 2014 11 29 09 1455-1475 |
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10.15252/emmm.201404436 doi (DE-627)SPR05795786X (SPR)emmm.201404436-e DE-627 ger DE-627 rakwb eng Falcone, Sestina verfasserin aut N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Authors. Published under the terms of the CC BY 4.0 license 2014 Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 Roman, William verfasserin aut Hnia, Karim verfasserin aut Gache, Vincent verfasserin aut Didier, Nathalie verfasserin aut Lainé, Jeanne verfasserin aut Auradé, Frederic verfasserin aut Marty, Isabelle verfasserin aut Nishino, Ichizo verfasserin aut Charlet‐Berguerand, Nicolas verfasserin aut Romero, Norma Beatriz verfasserin aut Marazzi, Giovanna verfasserin aut Sassoon, David verfasserin aut Laporte, Jocelyn verfasserin aut Gomes, Edgar R verfasserin aut Enthalten in EMBO Molecular Medicine Nature Publishing Group UK, 2023 6(2014), 11 vom: 29. Sept., Seite 1455-1475 (DE-627)594772761 (DE-600)2485479-7 1757-4684 nnns volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 https://dx.doi.org/10.15252/emmm.201404436 X:SPRINGER Resolving-System kostenfrei Volltext SYSFLAG_0 GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_72 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4029 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4116 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4155 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4311 GBV_ILN_4313 GBV_ILN_4314 GBV_ILN_4315 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4598 GBV_ILN_4700 AR 6 2014 11 29 09 1455-1475 |
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10.15252/emmm.201404436 doi (DE-627)SPR05795786X (SPR)emmm.201404436-e DE-627 ger DE-627 rakwb eng Falcone, Sestina verfasserin aut N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy 2014 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier © The Authors. Published under the terms of the CC BY 4.0 license 2014 Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 Roman, William verfasserin aut Hnia, Karim verfasserin aut Gache, Vincent verfasserin aut Didier, Nathalie verfasserin aut Lainé, Jeanne verfasserin aut Auradé, Frederic verfasserin aut Marty, Isabelle verfasserin aut Nishino, Ichizo verfasserin aut Charlet‐Berguerand, Nicolas verfasserin aut Romero, Norma Beatriz verfasserin aut Marazzi, Giovanna verfasserin aut Sassoon, David verfasserin aut Laporte, Jocelyn verfasserin aut Gomes, Edgar R verfasserin aut Enthalten in EMBO Molecular Medicine Nature Publishing Group UK, 2023 6(2014), 11 vom: 29. Sept., Seite 1455-1475 (DE-627)594772761 (DE-600)2485479-7 1757-4684 nnns volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 https://dx.doi.org/10.15252/emmm.201404436 X:SPRINGER Resolving-System kostenfrei Volltext SYSFLAG_0 GBV_SPRINGER GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_72 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_171 GBV_ILN_206 GBV_ILN_213 GBV_ILN_224 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_636 GBV_ILN_2001 GBV_ILN_2003 GBV_ILN_2004 GBV_ILN_2005 GBV_ILN_2006 GBV_ILN_2007 GBV_ILN_2009 GBV_ILN_2010 GBV_ILN_2011 GBV_ILN_2014 GBV_ILN_2015 GBV_ILN_2020 GBV_ILN_2021 GBV_ILN_2025 GBV_ILN_2026 GBV_ILN_2027 GBV_ILN_2034 GBV_ILN_2037 GBV_ILN_2038 GBV_ILN_2044 GBV_ILN_2048 GBV_ILN_2049 GBV_ILN_2050 GBV_ILN_2055 GBV_ILN_2056 GBV_ILN_2057 GBV_ILN_2059 GBV_ILN_2061 GBV_ILN_2064 GBV_ILN_2068 GBV_ILN_2088 GBV_ILN_2093 GBV_ILN_2106 GBV_ILN_2108 GBV_ILN_2110 GBV_ILN_2111 GBV_ILN_2118 GBV_ILN_2122 GBV_ILN_2129 GBV_ILN_2143 GBV_ILN_2144 GBV_ILN_2147 GBV_ILN_2148 GBV_ILN_2152 GBV_ILN_2153 GBV_ILN_2190 GBV_ILN_2232 GBV_ILN_2336 GBV_ILN_2470 GBV_ILN_2507 GBV_ILN_2522 GBV_ILN_2548 GBV_ILN_4012 GBV_ILN_4029 GBV_ILN_4035 GBV_ILN_4037 GBV_ILN_4046 GBV_ILN_4112 GBV_ILN_4116 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4155 GBV_ILN_4242 GBV_ILN_4246 GBV_ILN_4249 GBV_ILN_4251 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4311 GBV_ILN_4313 GBV_ILN_4314 GBV_ILN_4315 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4326 GBV_ILN_4328 GBV_ILN_4333 GBV_ILN_4334 GBV_ILN_4335 GBV_ILN_4336 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4393 GBV_ILN_4598 GBV_ILN_4700 AR 6 2014 11 29 09 1455-1475 |
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Enthalten in EMBO Molecular Medicine 6(2014), 11 vom: 29. Sept., Seite 1455-1475 volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 |
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Enthalten in EMBO Molecular Medicine 6(2014), 11 vom: 29. Sept., Seite 1455-1475 volume:6 year:2014 number:11 day:29 month:09 pages:1455-1475 |
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Falcone, Sestina @@aut@@ Roman, William @@aut@@ Hnia, Karim @@aut@@ Gache, Vincent @@aut@@ Didier, Nathalie @@aut@@ Lainé, Jeanne @@aut@@ Auradé, Frederic @@aut@@ Marty, Isabelle @@aut@@ Nishino, Ichizo @@aut@@ Charlet‐Berguerand, Nicolas @@aut@@ Romero, Norma Beatriz @@aut@@ Marazzi, Giovanna @@aut@@ Sassoon, David @@aut@@ Laporte, Jocelyn @@aut@@ Gomes, Edgar R @@aut@@ |
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Published under the terms of the CC BY 4.0 license 2014</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology.</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins.</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. 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Falcone, Sestina |
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Falcone, Sestina misc centronuclear myopathy misc cytoskeleton misc nuclear movement misc triad formation N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
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N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy centronuclear myopathy (dpeaa)DE-He213 cytoskeleton (dpeaa)DE-He213 nuclear movement (dpeaa)DE-He213 triad formation (dpeaa)DE-He213 |
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N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
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N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
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Falcone, Sestina Roman, William Hnia, Karim Gache, Vincent Didier, Nathalie Lainé, Jeanne Auradé, Frederic Marty, Isabelle Nishino, Ichizo Charlet‐Berguerand, Nicolas Romero, Norma Beatriz Marazzi, Giovanna Sassoon, David Laporte, Jocelyn Gomes, Edgar R |
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n‐wasp is required for amphiphysin‐2/bin1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
| title_auth |
N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
| abstract |
Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. © The Authors. Published under the terms of the CC BY 4.0 license 2014 |
| abstractGer |
Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. © The Authors. Published under the terms of the CC BY 4.0 license 2014 |
| abstract_unstemmed |
Abstract Mutations in amphiphysin‐2/BIN1, dynamin 2, and myotubularin are associated with centronuclear myopathy (CNM), a muscle disorder characterized by myofibers with atypical central nuclear positioning and abnormal triads. Mis‐splicing of amphiphysin‐2/BIN1 is also associated with myotonic dystrophy that shares histopathological hallmarks with CNM. How amphiphysin‐2 orchestrates nuclear positioning and triad organization and how CNM‐associated mutations lead to muscle dysfunction remains elusive. We find that N‐WASP interacts with amphiphysin‐2 in myofibers and that this interaction and N‐WASP distribution are disrupted by amphiphysin‐2 CNM mutations. We establish that N‐WASP functions downstream of amphiphysin‐2 to drive peripheral nuclear positioning and triad organization during myofiber formation. Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. In adult myofibers, N‐WASP and amphiphysin‐2 are only involved in the maintenance of triad organization but not in the maintenance of peripheral nuclear positioning. Importantly, we confirmed that N‐WASP distribution is disrupted in CNM and myotonic dystrophy patients. Our results support a role for N‐WASP in amphiphysin‐2‐dependent nuclear positioning and triad organization and in CNM and myotonic dystrophy pathophysiology. Synopsis Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. Amphiphysin‐2/BIN1 interacts with N‐WASP in skeletal muscle.Amphiphysin‐2/BIN1 and N‐WASP are required for peripheral nuclei positioning and triad formation.Peripheral nuclear positioning requires microtubule/Map7/Kif5b‐dependent distribution of nuclei along the myofiber and is driven by actin and nesprins. Graphical Abstract Amphiphysin‐2/BIN1 is known to associate with centronuclear myopathy (CNM) and myotonic dystrophy. N‐WASP is found downstream of Amphiphysin‐2/BIN1 and aberrantly distributed in skeletal muscle of patients. Activation of N‐WASP could provide a therapeutic option for CNM. © The Authors. Published under the terms of the CC BY 4.0 license 2014 |
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N‐WASP is required for Amphiphysin‐2/BIN1‐dependent nuclear positioning and triad organization in skeletal muscle and is involved in the pathophysiology of centronuclear myopathy |
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Roman, William Hnia, Karim Gache, Vincent Didier, Nathalie Lainé, Jeanne Auradé, Frederic Marty, Isabelle Nishino, Ichizo Charlet‐Berguerand, Nicolas Romero, Norma Beatriz Marazzi, Giovanna Sassoon, David Laporte, Jocelyn Gomes, Edgar R |
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| score |
7.4000454 |