Journal: Antioxidants
Article Title: Mitochondrial Unfolded Protein Response (mtUPR) Activation Improves Pathological Alterations in Cellular Models of Ethylmalonic Encephalopathy
doi: 10.3390/antiox14060741
Figure Lengend Snippet: Effect of CoC3 treatment for seven days on the expression levels of proteins of the transcriptional canonical mtUPR axis. ( A ) Cellular extracts from untreated and treated with CoC3 control (C1 and C2) and fibroblasts derived from the EE patient (P) fibroblasts were analysed by Western blot analysis. The membranes were immunostained using antibodies against eif2α, P-eif2α, ATF4, ATF5, HSP60, HSP70, and β-actin, the latter used as a loading control. ( B ) Densitometry of Western blotting. Untreated and treated control samples were unified in one value for each condition (C and C + CoC3, respectively) which represents the mean of each control measurement. Densitometry was referred to the untreated control (C) value. Data were referred to the control and represent the mean ± SD of 3 independent experiments. ** p < 0.01 and *** p < 0.001 between control and patient fibroblasts. aa p < 0.01, and aaaa p < 0.0001 between untreated and treated patient fibroblasts. a.u.: arbitrary units.
Article Snippet: Anti-eukaryotic translation initiation factor 2α (eif2α) (9722S), anti-phospho-eif2α (9721S), anti-mitochondrially encoded NADH Dehydrogenase Subunit 1 (mt-ND1) (6888S), anti-mitochondrial transcription factor A (TFAM) (7495S), and anti-Activating Transcription Factor 4 (ATF4) (11815S) were purchased from Cell Signaling (Danvers, MA, USA).
Techniques: Expressing, Control, Derivative Assay, Western Blot