nemoci-sympt/METABOLISMUS/mitochondrie/metabolizmus/volne-radikaly
Volné radikály
Superoxide (O22 -)
- Produced at several sites in the matrix and intermembrane space:
- Flavin moiety of complex I
- Ubiquinone-binding sites in complex III
- Glycerol 3-phosphate dehydrogenase
- Electron transferring flavoprotein:Q oxidoreductase (ETFQOR) of fatty acids
- Branched-chainamino acid oxidation
- Pyruvate dehydrogenase
- 2-oxoglutarate dehydrogenase [9]
- Rapidly converted by the mitochondrial manganese superoxide dismutase (SOD2) into:
- hydrogen peroxide (H2O2)
- Much less harmful
- Superoxide produced in the intermembrane space
- Can exit the mitochondria in the cytosol
- Converted by copper superoxide dismutase (SOD1) into
H2O2
- Can diffuse through inner and outer mitochondrial membranes
- Access the cytosol
- Main form of ROS with signaling function in the cell
- Can oxidize protein thiol residues
- Can be converted to water by:
- mitochondrial glutathione peroxidases (GPX)
- Peroxiredoxins (PRX) [9]
- Conversion by:
- Peroxisomal catalase
- Increased ROS production
- Occurs as a consequence of:
- Respiratory chain dysfunction due to
- Aging
- Specific OXPHOS defects
- May lead to:
- Damage of cellular structures
- Rational basis for the use of antioxidants in the therapy of mitochondrial diseases
- ROS can transduce signals in a number of pathways
- Cocktails of antioxidant compounds extensively been used in the therapy of mitochondrial diseases
- Lipoic acid, vitamins C and E, and CoQ
- no quantitative studies have been carried out
- Randomized double blind trials are still missing to support their efficacy in patients
- Transgenic mouse overexpressing a mitochondrially-targeted catalase
- Increased lifespan and resistance to oxidative damage
- Importance of ROS overproduction in the pathogenesis of cI-related Leigh syndrome
- Potential therapeutic target in cI-related disorders in cell models [9]
Volné radikály
Superoxide (O22 -)
- Produced at several sites in the matrix and intermembrane space:
- Flavin moiety of complex I
- Ubiquinone-binding sites in complex III
- Glycerol 3-phosphate dehydrogenase
- Electron transferring flavoprotein:Q oxidoreductase (ETFQOR) of fatty acids
- Branched-chainamino acid oxidation
- Pyruvate dehydrogenase
- 2-oxoglutarate dehydrogenase [9]
- Rapidly converted by the mitochondrial manganese superoxide dismutase (SOD2) into:
- hydrogen peroxide (H2O2)
- Much less harmful
- Superoxide produced in the intermembrane space
- Can exit the mitochondria in the cytosol
- Converted by copper superoxide dismutase (SOD1) into
H2O2
- Can diffuse through inner and outer mitochondrial membranes
- Access the cytosol
- Main form of ROS with signaling function in the cell
- Can oxidize protein thiol residues
- Can be converted to water by:
- mitochondrial glutathione peroxidases (GPX)
- Peroxiredoxins (PRX) [9]
- Conversion by:
- Peroxisomal catalase
- Increased ROS production
- Occurs as a consequence of:
- Respiratory chain dysfunction due to
- Aging
- Specific OXPHOS defects
- May lead to:
- Damage of cellular structures
- Rational basis for the use of antioxidants in the therapy of mitochondrial diseases
- ROS can transduce signals in a number of pathways
- Cocktails of antioxidant compounds extensively been used in the therapy of mitochondrial diseases
- Lipoic acid, vitamins C and E, and CoQ
- no quantitative studies have been carried out
- Randomized double blind trials are still missing to support their efficacy in patients
- Transgenic mouse overexpressing a mitochondrially-targeted catalase
- Increased lifespan and resistance to oxidative damage
- Importance of ROS overproduction in the pathogenesis of cI-related Leigh syndrome
- Potential therapeutic target in cI-related disorders in cell models [9]
