Additional file 1 of Severe ACTA1-related nemaline myopathy: intranuclear rods, cytoplasmic bodies, and enlarged perinuclear space as characteristic pathological features on muscle biopsies

Additional file 1 of Severe ACTA1-related nemaline myopathy: intranuclear rods, cytoplasmic bodies, and enlarged perinuclear space as characteristic pathological features on muscle biopsies

Additional file 1: Figure S1. Myofibre nuclear envelope line scan profiles. Quantification of signal intensities at the middle of the nuclei shows an abnormal lamin A/C, Nesprin-1, and Nesprin-2 localization in patients 6 and 7 compared with the age-matched control. Red brackets show the extension o...

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Hauptverfasser: Labasse, Clémence, Brochier, Guy, Taratuto, Ana-Lia, Cadot, Bruno, Rendu, John, Monges, Soledad, Biancalana, Valérie, Quijano-Roy, Susana, Bui, Mai Thao, Chanut, Anaïs, Madelaine, Angéline, Lacène, Emmanuelle, Beuvin, Maud, Amthor, Helge, Servais, Laurent, de Feraudy, Yvan, Erro, Marcela, Saccoliti, Maria, Neto, Osorio Abath, Fauré, Julien, Lannes, Béatrice, Laugel, Vincent, Coppens, Sandra, Lubieniecki, Fabiana, Bello, Ana Buj, Laing, Nigel, Evangelista, Teresinha, Laporte, Jocelyn, Böhm, Johann, Romero, Norma B.
Format: Bild
Sprache:eng
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FOS: Biological sciences
Genetics
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Zusammenfassung:Additional file 1: Figure S1. Myofibre nuclear envelope line scan profiles. Quantification of signal intensities at the middle of the nuclei shows an abnormal lamin A/C, Nesprin-1, and Nesprin-2 localization in patients 6 and 7 compared with the age-matched control. Red brackets show the extension of nuclear envelope proteins into the cytoplasm. Each graph corresponds to one nucleus. Bottom right: explanatory scheme of the analysis with the nucleoplasm depicted in grey.
DOI:10.6084/m9.figshare.20282184