Figure 1.
Several examples of maladaptive GSH responses in lung disease states. In Cystic Fibrosis lung disease, infections drive GSH adaptive responses, yet due to the CFTR mutation there is an inability to transport GSH into the ELF. In COPD and aging, chronic cigarette smoke drives GSH adaptive responses, however aging adversely affects Nrf2 activation which leads to deficient enzyme production and decreased intracellular GSH and well as decreased ELF GSH. In lung fibrosis, exaggerated TGF-β production in the airways produces affects on poorly identified transcriptional events leading to decreased γGCL levels and GSH synthesis.