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. 2018 Jul 1;198(1):90–103. doi: 10.1164/rccm.201708-1751OC

Figure 3.

Figure 3.

Mitochondrial HSP90 controls mitochondrial DNA (mtDNA) integrity. (A) Quantification of baseline mtDNA damage per 10-kb DNA by a semi-long quantitative PCR amplification of total DNA isolated from control (n = 4) and pulmonary arterial hypertension–pulmonary artery smooth muscle cells (PAH-PASMCs) (n = 4). (B) Quantification of mtDNA lesions in PAH-PASMCs (n = 4) exposed to 2 μM Gamitrinib or its vehicle (DMSO) for 48 hours. (C) Effects of Gamitrinib on mtDNA copy number in PAH-PASMCs (n = 4). (D) Representative Western blots and corresponding densitometric analyses of OGG1 (8-oxoguanine DNA glycosylase-1), LIG3 (DNA ligase 3), POLG1 (polymerase γ), and TFAM (mitochondrial transcription factor A) expression in PASMCs isolated from control subjects (n = 5) and patients with PAH (n = 7). (E) Representative Western blots and corresponding densitometric analyses of POLG1, OGG1, TFAM, and LIG3 expression in PAH-PASMCs (n = 6) treated or not with Gamitrinib (2 μM) or its vehicle (DMSO) for 48 hours. Protein expression was normalized to Amido black (AB). Data are presented as mean ± SEM; *P < 0.05; **P < 0.01. CTRL = control; Gami = Gamitrinib; nb = number; NT = nontreated; Veh = vehicle.