[HTML][HTML] An intracellular serpin regulates necrosis by inhibiting the induction and sequelae of lysosomal injury

CJ Luke, SC Pak, YS Askew, TL Naviglia, DJ Askew… - Cell, 2007 - cell.com
CJ Luke, SC Pak, YS Askew, TL Naviglia, DJ Askew, SM Nobar, AC Vetica, OS Long…
Cell, 2007cell.com
Extracellular serpins such as antithrombin and α1-antitrypsin are the quintessential
regulators of proteolytic pathways. In contrast, the biological functions of the intracellular
serpins remain obscure. We now report that the C. elegans intracellular serpin, SRP-6,
exhibits a prosurvival function by blocking necrosis. Minutes after hypotonic shock, srp-6 null
animals underwent a catastrophic series of events culminating in lysosomal disruption,
cytoplasmic proteolysis, and death. This newly defined hypo-osmotic stress lethal (Osl) …
Summary
Extracellular serpins such as antithrombin and α1-antitrypsin are the quintessential regulators of proteolytic pathways. In contrast, the biological functions of the intracellular serpins remain obscure. We now report that the C. elegans intracellular serpin, SRP-6, exhibits a prosurvival function by blocking necrosis. Minutes after hypotonic shock, srp-6 null animals underwent a catastrophic series of events culminating in lysosomal disruption, cytoplasmic proteolysis, and death. This newly defined hypo-osmotic stress lethal (Osl) phenotype was dependent upon calpains and lysosomal cysteine peptidases, two in vitro targets of SRP-6. By protecting against both the induction of and the lethal effects from lysosomal injury, SRP-6 also blocked death induced by heat shock, oxidative stress, hypoxia, and cation channel hyperactivity. These findings suggest that multiple noxious stimuli converge upon a peptidase-driven, core stress response pathway that, in the absence of serpin regulation, triggers a lysosomal-dependent necrotic cell death routine.
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