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The parents of an individual with an autosomal recessive disorder each carry one copy of the defective gene, but usually are not affected by the disorder

Eighty-five to ninety percent of cellular glutathione is present in the cytosol, and rest 10-15% is distributed in many intracellular organelles like mitochondria, nuclear matrix, endoplasmic reticulum (ER), peroximes and nucleus.[26,27,118] Concentration of mitochondrial glutathione is similar to that of the cytosol (10-14 mM) by the volume of the mitochondrial matrix.[118] Its concentration in the human liver tissue is evaluated as 6,400 mmol/kg.[117] Relatively low (2-20 mol/L in plasma) concentration of glutathione is observed in the extracellular environment, whereas, bile acids may contain up to 10 mmol/L glutathione.[27] Glutathione concentration is found to be greatest in the liver, the organ involved in detoxification and elimination of toxins, and in the lung epithelial lining fluid (ELF) (of about 400 M), which is raised in the case of chronic smokers and lung cancer patients,[34,117] and decreased in patients with pulmonary fibrosis and acute respiratory distress syndrome.[34] The AO properties of glutathione are mediated by redox-active thiol group that becomes oxidised when glutathione reduces target molecules, which may be attributed to the fact that glutathione displays a low redox potential (E0 = 240 mV), and is found in high concentration in the cells (approximately 10-15 Mm).[118] In the presence of electrophilic substances, e.g., free radicals and ROS/nitrogen sps, glutathione readily oxidizes non-enzymatically to glutathione disulfide (GSSG).[27] As glutathione acts as electron carriers, so when electrons are lost by oxidation, glutathione becomes oxidized (GS ), and two such molecules dimerized by a disulfide bridge to form disulfide glutathione or oxidized glutathione (GSSG)

For example, hepatotoxicity may be amplified not only by 17-alkylated oral steroids but also by concomitant use of other hepatically metabolized agents, alcohol, contaminated or counterfeit products, and high-dose supplements (93, 95)

Interestingly, GGsTop similarly suppressed TNF, MCP-1 and MUC5AC in the lung of OVA/LPS/GGsTop treated mice ( Figure 3A )

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Senotherapeutics: Emerging Strategy for Healthy Aging and Age-Related Disease. BMB Reports, vol