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1b): 7,8,9,10,11,12,15,17,19,30 During the oxidative half-reaction the thiolate group of the reduced glutaredoxin attacks the GSSR disulfide bond, yielding the first product (RSH) and the glutathionylated glutaredoxin Grx(SSG) as an intermediate

In addition to the findings with ASMase knockout mice, which models the lysosomal storage disorder NiemannPick type A disease, inhibition of glucocerebrosidase in hepatocytes by conduritol -epoxide, a cellular model of Gaucher disease that exhibits lysosomal cholesterol accumulation sensitizes to APAP-induced cell death (Teratani et al., 2017)

Within the context of the TCA cycle the acetyl-CoA generated by the PDHc is completely oxidized to CO 2 and H 2 O