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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

From: rAAV-related therapy fully rescues myonuclear and myofilament function in X-linked myotubular myopathy

Fig. 5

Delivery of wild type MTM1 gene rescues disrupted histone acetylation in XLMTM dogs. acetyl histone H3 (Lys9/Lys14) (referred to her as AcH3) is a histone modification marking actively transcribed regions of DNA. Mean fluorescence intensity of immunolabelled AcH3 correlates with global gene transcription levels [22, 31]. Dogs from 5 groups were analysed (N = 3 per group, see legend from Fig. 2): Healthy, XLMTM, AAVLow, AAVMid, and AAVHigh. a Representative single myonuclei from each group, stained for AcH3 (red). Scale bar: 10 μm. b Mean AcH3 intensity calculated for 10 nuclei per fibre. c Mean standard deviation of pixel intensity, as a percentage of mean pixel intensity, of AcH3 staining within individual nuclei. This is a measure homogeneity of immunolabelling within each nucleus, and suggests more irregular distribution of chromatin and/or active regions of DNA in XLMTM and AAVLow myonuclei. Data points correspond to individual muscle fibres (10 nuclei per fibre, from a total of 12 fibres per treatment group). Column graphs show mean ± S.D. Asterisks denote a significant difference compared with CTL (* P < 0.05; ** P < 0.01; one-way ANOVA)

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