Fig. 2: Functional and expression validation of proteomic data. | Cell Death Discovery

Fig. 2: Functional and expression validation of proteomic data.

From: Proteomic and functional analyses in disease models reveal CLN5 protein involvement in mitochondrial dysfunction

Fig. 2

a Validation of protein expression levels related to selected mtDEPs, assessed in HEK 293T and SH-SY5Y whole cell lysates. Densitometric analyses corroborate the results of mitochondrial proteome profiling. b Analysis of mitochondrial energy metabolism in neuroblastoma cells CLN5 KO using the Seahorse XF Cell Mito Stress Test, shows a decreased basal oxygen consumption rate as well as maximal respiration whereas no significant differences were observed in non-mitochondrial respiration. c Spectrophotometric determination of RC enzymes in cerebral cortex from symptomatic mice reveals a general OXPHOS impairment with a significant reduction of complexes III and I + III and increase of citrate synthase activity as index of mitochondrial proliferation. Data represent the mean (± standard deviation) of three independent experiments (n = 9). Statistical significance was determined by two-tailed Student t test *p < 0.05; **p < 0.01; ***p < 0.001.

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