2020
DOI: 10.3389/fcell.2020.610266
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Relationship Between Mitochondrial Structure and Bioenergetics in Pseudoxanthoma elasticum Dermal Fibroblasts

Abstract: Pseudoxanthoma elasticum (PXE) is a genetic disease considered as a paradigm of ectopic mineralization disorders, being characterized by multisystem clinical manifestations due to progressive calcification of skin, eyes, and the cardiovascular system, resembling an age-related phenotype. Although fibroblasts do not express the pathogenic ABCC6 gene, nevertheless these cells are still under investigation because they regulate connective tissue homeostasis, generating the “arena” where cells and extracellular ma… Show more

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Cited by 15 publications
(16 citation statements)
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“…ROS are generated by different sources such as NADPH oxidase, xanthine oxidase, uncoupled endothelial nitric-oxide synthase, and mitochondria. We recently demonstrated in PXE increased mitochondrial ROS associated with mitochondrial dysfunction (e.g., increased mitochondrial membrane potential, altered mitochondrial morphology, low free calcium within mitochondria) [ 40 ]. Therefore, the activation of BMP signalling, observed in PXE, may be favored by oxidative stress.…”
Section: Resultsmentioning
confidence: 99%
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“…ROS are generated by different sources such as NADPH oxidase, xanthine oxidase, uncoupled endothelial nitric-oxide synthase, and mitochondria. We recently demonstrated in PXE increased mitochondrial ROS associated with mitochondrial dysfunction (e.g., increased mitochondrial membrane potential, altered mitochondrial morphology, low free calcium within mitochondria) [ 40 ]. Therefore, the activation of BMP signalling, observed in PXE, may be favored by oxidative stress.…”
Section: Resultsmentioning
confidence: 99%
“…Therefore, TGF-β-SMAD2/3 activation in PXE is, at least in part, related to oxidative stress [ 37 , 40 ] and to MMPs activity [ 18 , 19 ].…”
Section: Resultsmentioning
confidence: 99%
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“…Proteins identified only in one sample per condition were excluded by further analysis. We performed label-free quantification using a tandem mass spectra counting approach [44], as already described [45,46]. Briefly, the exponentially modified protein abundance index (emPAI), integrated into Mascot, was calculated as the ratio between the number of experimentally observed peptides per protein and the number of theoretically observable peptides per protein.…”
Section: Discussionmentioning
confidence: 99%
“…Cells at confluence were detached with scraper and embedded as already described [ 39 ]. Ultrathin sections (60 nm) were cut and mounted on 150 mesh copper grids (Electron Microscopy Sciences, Hatfield, PA, USA).…”
Section: Methodsmentioning
confidence: 99%