FIGURE

Fig. 5

ID

ZDB-FIG-201208-15

Publication

Gut et al., 2020 - SUCLA2 mutations cause global protein succinylation contributing to the pathomechanism of a hereditary mitochondrial disease

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Fig. 5

Sirt5 restores oxidative metabolism and improves survival of <italic>sucla2</italic><sup>−/−</sup> zebrafish.

a Oxygen consumption rate (OCR) in sucla2^−/− and control zebrafish larvae with or without sirt5 overexpression (n = 15 per group, 10dpf), before and after the addition of inhibitors of mitochondrial respiration. The uncoupler FCCP is added to stimulate maximal OCR. Rotenone and antimycin A (R + A) determine non-mitochondrial respiration. b Basal OCR calculating the average of the three last points before addition of FCCP. c maximal OCR. d qPCR analysis of mitochondrial DNA content in sucla2^−/− and control zebrafish larvae with or without sirt5 overexpression (n = 12 per group, 10dpf). e Survival of sucla2^−/− and control zebrafish larvae with or without sirt5 overexpression in standard (fed) conditions (sucla2^+/+, n = 40; sucla2^−/−, n = 37; sucla2^+/+;Tg(ubi:sirt5), n = 38; sucla2^−/−;Tg(ubi:sirt5), n = 36). Data are pooled from two independent experiments. p-Value for median survival of wild type vs sucla2^−/− <0.0001, p-value sucla2^−/− vs sucla2^−/−;Tg(ubi:sirt5) = 0.00061. Pairwise comparison using log-rank test. p-Value adjustment was performed by Bonferroni correction. The boxplots show the median, the first to third quartile, and minima and maxima). n.s., not significant. mtDNA = mitochondrial DNA; dpf = days post fertilization. Source data are provided as a Source Data file.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	nei005Tg/nei005Tg sucla2^{nei010/nei010} sucla2^{nei010/nei010}; nei005Tg/nei005Tg
Condition:	chemical treatment by environment: carbonyl cyanide p-trifluoromethoxyphenylhydrazone
Observed In:	aerobic respiration whole organism whole organism mitochondrial chromosome
Stage Range:	Protruding-mouth to Days 7-13

Phenotype Detail

Acknowledgments

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