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Figure 3 Mutation carrier–derived lymphoblastoid cell lines (LCLs) show decreased aconitase 2 activity and mitochondrial respiration deficiency compared.

Published byFabien Dumont Modified over 5 years ago

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Presentation on theme: "Figure 3 Mutation carrier–derived lymphoblastoid cell lines (LCLs) show decreased aconitase 2 activity and mitochondrial respiration deficiency compared."— Presentation transcript:

1 Figure 3 Mutation carrier–derived lymphoblastoid cell lines (LCLs) show decreased aconitase 2 activity and mitochondrial respiration deficiency compared with control LCLs Mutation carrier–derived lymphoblastoid cell lines (LCLs) show decreased aconitase 2 activity and mitochondrial respiration deficiency compared with control LCLs (A) Aconitase enzyme activity in LCL lysates of healthy controls (ACOc/c) and homozygous patient carriers (ACO2F414V/F414V) (p = 1.6 × 10−8). (B) Western blot of corresponding LCL lysates showing equal ACO2 protein levels in healthy controls and homozygous carrier patients. (C) Mitochondrial fraction of LCL lysates show a significant decrease in aconitase enzyme activity in homozygous patient carrier samples (p = 7.4 × 10−7). (D) ACO2 enzyme activity is unchanged in the cytoplasmic fraction of corresponding LCL lysates (p = 0.11). Data in (A, C and D) are expressed as mean ± SEM, representative experiment of 3 independent experiments, n = 6 time curve measurements per condition; results of the paired t test are indicated. *p < Christian G. Bouwkamp et al. Neurol Genet 2018;4:e223 Copyright © 2018 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology

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