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Trichothiodystrophy (TTD) is a rare hereditary disease whose prominent feature is brittle hair. Additional clinical signs are physical and neurodevelopmental abnormalities and in about half of the cases hypersensitivity to UV radiation. Although the mutations involved in this condition have been characterized, the correlation between the molecular defects and the plethora of clinical symptoms is not well understood. Recently the presence of a redox unbalance in TTD has been suggested although not clear evidences have been reported on this aspect.
Therefore, in the present study, we evaluated the redox status of fibroblasts isolated from a TTD patient. In addition, to understand the ability of TTD cells to respond to oxidative insults, the cells were challenged with H2O2 and mitochondrial O2- and mitochondrial membrane potential were measured in different oxidative conditions. In addition, NRF2/BACH protein levels were also analysed in response to H2O2.
The results suggested an aberrant mitochondrial response to oxidative stimuli, an increased baseline oxidative stress status in TTD and an altered NRF2/BACH level.
This study emphasises that altered redox homeostasis might play a role in TTD pathogenesis and mitochondria functionality could represent an alternative therapeutic target for this condition to improve patients clinical features.
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the modulation of hepatic regenerative homeostasis, pointing out the most important clinical perspectives. The hepatic redox homeostasis The liver accounts for the metabolism of carbohydrates, lipids, and ammonia, biotransformation of
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role in the removal of reactive oxygen species (ROS) and redox regulation. Selenoproteins are also a key factor in the antioxidant system ( Conrad et al. 2018 , Dagnell et al. 2018 ). Although selenium is an essential element, it is highly toxic
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Department of Environmental Toxicology, University of California, Davis, California, USA
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homeostasis, and in supporting relevant intestinal epithelium functions, e.g. intestinal barrier physiology. Physiological redox homeostasis is attained by regulating the production of oxidant species, managing their removal, and through the repair of oxidized
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Department of Pharmacology and Toxicology, Virginia Commonwealth University, Richmond, Virginia, USA
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Department of Pharmacology and Toxicology, Virginia Commonwealth University, Richmond, Virginia, USA
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Department of Pharmacology, College of Graduate Studies, Midwestern University, Downers Grove, Illinois, USA
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redox potential (Rosales, 2014 #121). Glutathione by capturing the excess reactive nitric oxide species from respective proteins which are nitrosylated tries to maintain the nitrosative stress homeostasis (Yoon, 2021 #117; Rosales, 2014 #121; Larrick
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involved in eNOS dimerization, which could be impaired by S-nitrosylation ( Erwin et al. 2005 , 2006 ). Oxidative stress and ONOO − trigger eNOS uncoupling in CVD In vascular cells and tissues, redox homeostasis is physiologically regulated
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Animal Science Department, Plants for Human Health Institute, North Carolina State University, Kannapolis Research Campus, Kannapolis, North Carolina, USA
Department of Food and Nutrition, Kyung Hee University, Seoul, South Korea
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addition, at molecular level, RTT is characterized by an alteration of several metabolic traits, such as redox homeostasis, mitochondrial bioenergetics, cholesterol metabolism and inflammatory responses ( Sticozzi et al. 2013 , Segatto et al. 2014
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potentially play in adaptations to contractile activity. Some of these beneficial adaptations of muscle to exercise or contractile activity are also attenuated during aging and this appears particularly relevant for redox-regulated pathways. The review will
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Introduction Glutathione (GSH) is mainly present in the cytosol, and its distribution between the intracellular compartments is crucial to regulate redox homeostasis, gene expression, cell signaling and proliferation ( Sies 1999 ). The
PULSALYS SATT Lyon-Saint Etienne, Villeurbanne, France
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cellular redox homeostasis by an elaborate endogenous antioxidant defense system ( Fig. 1 ). Figure 1 Representative illustration of the cellular redox homeostasis balanced from antioxidant and ROS levels. The first line of defense against
