Reactive Oxygen Species and Oxidative Stress in the Pathogenesis and Progression of Genetic Diseases of the Connective Tissue

We review the impact of reactive oxygen species and oxidative stress on the onset and/or progression of diseases that directly affect connective tissue and have a genetic origin

Gustavo Egea; Francesc Jiménez-Altayó; Victoria Campuzano

2020

Scholarcy highlights

  • Connective tissue is the body’s structural support and a dynamic site for other important functions
  • Basal protein kinase G activity was significantly increased in mice carrying the protein kinase G1 mutation, which leads to oxidative stress, increased vascular SMCs apoptosis, and elastin fiber breaks
  • Radical species generated at cellular level have a great impact on cellular components, whose functions can significantly change in the short or long term depending on how long the radicals are present in the cell environment
  • It is evident that when radicals are constantly produced and exceed the buffering capacity of endogenous antioxidants, the physiological role of reactive oxygen species becomes detrimental, which leads to oxidative stress
  • We examined the impact of ROS and oxidative stress in genetic diseases of CT
  • In Col4a3−/− hearts, oxidative stress was markedly elevated, including 50% reduction in the GSH:GSSG ratio, as well as reductions in the protein levels of the mitochondrial electron transport chain of complexes I, II, and IV and a 35% increase in malondialdehyde
  • ROS and oxidative stress are involved in the CT pathology of different genetic diseases at molecular and cellular levels
  • Pharmacological interference of oxidative stress in genetic diseases that affect Connective tissue formation deserves more attention

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