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Fig. 5 | Acta Neuropathologica Communications

Fig. 5

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

Fig. 5

Quantification of the phenotypic severity of neb−/− mutants at 6 dpf. Quantification of the phenotypic severity of Tg(neb−/−; Lifeact-eGFP) fish at 6 dpf supplemented with either L-tyrosine, taurine, L-carnitine, creatine, or water (H2O). a Phenotypes were scored as either wildtype, mild (less than five Lifeact-eGFP positive aggregates at the myosepta or a mild disruption of muscle fibres), or severe (severely disorganised fibres or an accumulation of five or more Lifeact-eGFP positive aggregates within the muscle cell). b Quantification of the phenotypic severity of Tg(neb−/−; Lifeact-eGFP) fish supplemented with either L-tyrosine, taurine, or water. c Quantification of the phenotypic severity of Tg(neb−/−; Lifeact-eGFP) fish supplemented with either L-carnitine, creatine, or water (H2O). b & c Error bars represent mean±SEM for three independent experiments. For b) n = 6,8,7 Tg(neb−/−; Lifeact-eGFP) for L-tyrosine, n = 11,5,11 Tg(neb−/−; Lifeact-eGFP) for taurine and n = 9,8,10 Tg(neb−/−; Lifeact-eGFP) for water. For c) n = 8,10,4 Tg(neb−/−; Lifeact-eGFP) for L-carnitine, n = 6,8,3 Tg(neb−/−; Lifeact-eGFP) for creatine, and n = 10,9,5 Tg(neb−/−; Lifeact-eGFP) for water per experiment). ns = not significant

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