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Fig. 1 | Acta Neuropathologica Communications

Fig. 1

From: Testing of therapies in a novel nebulin nemaline myopathy model demonstrate a lack of efficacy

Fig. 1

Characterization of the neb (sa906) mutant zebrafish strain. a & b) At 4 dpf neb−/− zebrafish (aii) appear smaller in size and (bii) display a loss of birefringence compared to their wildtype siblings (ai & bi). c) RT-PCR analysis in and neb−/− mutant embryos at 2 dpf shows a reduction in neb mRNA levels compared to wildtype siblings (sibling). βAct was used as a positive control. d) ii) neb−/− mutants display a smaller eye, brain region (B) and deflated swim bladder (SB) compared to their i) wildtype siblings. e) Quantification of the maximum acceleration recorded from touch-evoked response assays of neb−/− fish compared to wildtype siblings at 2 dpf. Error bars represent mean±SEM for three independent experiments (n = 11,9,12 neb−/−, 55,25,32 neb+/−, 18,13,12 neb+/+ zebrafish per experiment), **p < 0.01. f & g) Quantification of the normalized (f) distance and (g) speed travelled by neb−/− mutants compared to wildtype siblings at 6 dpf. For f) error bars represent median±interquartile range for three independent experiments (for n = 19,23,19 neb−/−, 41,42,36 neb+/−, 31,20,21 neb+/+ zebrafish). For g) error bars represent mean±SEM range (for n = 19,23,14 neb−/−, 41,42,36 neb+/−, 30,20,21 neb+/+ zebrafish per experiment). *p < 0.5, **** p < 0.001

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