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. 2021 Mar 12;143(21):2091–2109. doi: 10.1161/CIRCULATIONAHA.120.051171

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© 2021 The Authors.

Circulation is published on behalf of the American Heart Association, Inc., by Wolters Kluwer Health, Inc. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution, and reproduction in any medium, provided that the original work is properly cited.

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Figure 3. — Decrease of TFAM levels and mitochondrial respiration in samples from MFS patients. A, Analysis of human ascending aortic samples from MFS patients and control donors. B, Quantitative reverse transcription polymerase chain reaction analysis of TFAM mRNA and quantitative polymerase chain reaction analysis of relative mtDNA content. C, Expression of genes encoding mitochondrial complex components and (D) genes related to mitochondrial function and glycolysis. E, Representative medial layer sections of immunohistochemical analysis of MT-ND1 (CoI), SDHA (CoII), TFAM, HIF1A, and MYC levels and (F) quantification. G through K, Primary fibroblasts from 4 patients with MFS and 4 healthy controls. G, Quantitative polymerase chain reaction analysis of mtDNA content and quantitative reverse transcription polymerase chain reaction analysis of TFAM and PPARGC1A expression (H) and representative TFAM and PGC1α immunoblotting (n=4). I, mRNA of MT-ND1, SDHA, CYCS, MT-CO1, MT-ATP6, (J) UCP2, HIF1A, and MYC as assessed by quantitative reverse transcription polymerase chain reaction, in extracts from human MFS and Control fibroblast. K, OCR after addition of oligomycin (I), fluoro carbonyl cyanide phenylhydrazone (II), and a combination of rotenone and antimycin-A (III) and extracellular lactate levels. Data are mean±SEM. Statistical significance was assessed by Student t test *P<0.05, **P<0.01, ***P<0.001, ****P<0.0001 vs Control. ACTIN indicates beta actin; COI–V, mitochondrial complexes I-V; CYCS, Cytochrome C, Somatic; HIF1A, hypoxia-inducible factor 1 α; MFS, Marfan syndrome; MT-ATP6, mitochondrially-encoded ATP synthase membrane subunit 6; MT-CO1, mitochondrially-encoded cytochrome c oxidase I; MT-ND1, mitochondrially-encoded NADH ubiquinone oxidoreductase core subunit 1; MYC, MYC proto-oncogene, bHLH transcription factor; PPARA, Peroxisome proliferator-activated receptor alpha; PPARAGC, peroxisome proliferator-activated receptor γ coactivator; PPARAGC1a, Peroxisome proliferator-activated receptor gamma coactivator 1-alpha; PGC1α, Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (protein); SDHA, B, succinate dehydrogenase complex flavoprotein subunit A-B; TFAM, mitochondrial transcription factor A; and UCP2, mitochondrial uncoupling protein 2.