Nitrosative stress mediated misfolded protein aggregation mitigated by Na-d-β-hydroxybutyrate intervention

P Kabiraj, R Pal, A Varela-Ramirez, M Miranda… - Biochemical and …, 2012 - Elsevier
P Kabiraj, R Pal, A Varela-Ramirez, M Miranda, M Narayan
Biochemical and Biophysical Research Communications, 2012Elsevier
Mitochondrial dysfunction, leading to elevated levels of reactive oxygen species, is
associated with the pathogenesis of neurodegenerative disorders. Rotenone, a
mitochondrial stressor induces caspase-9 and caspase-3 activation leading proteolytic
cleavage of substrate nuclear poly (ADP-ribose) polymerase (PARP). PARP cleavage is
directly related to apoptotic cell death. In this study, we have monitored the aggregation of
green-fluorescent protein (GFP)-tagged synphilin-1, as a rotenone-induced Parkinsonia …
Mitochondrial dysfunction, leading to elevated levels of reactive oxygen species, is associated with the pathogenesis of neurodegenerative disorders. Rotenone, a mitochondrial stressor induces caspase-9 and caspase-3 activation leading proteolytic cleavage of substrate nuclear poly(ADP-ribose) polymerase (PARP). PARP cleavage is directly related to apoptotic cell death. In this study, we have monitored the aggregation of green-fluorescent protein (GFP)-tagged synphilin-1, as a rotenone-induced Parkinsonia-onset biomarker. We report that the innate ketone body, Na-d-β-hydroxybutyrate (NaβHB) reduces markedly the incidence of synphilin-1 aggregation. Furthermore, our data reveal that the metabolic byproduct also prevents rotenone-induced caspase-activated apoptotic cell death in dopaminergic SH-SY5Y cells. Together, these results suggest that NaβHB is neuroprotective; it attenuates effects originating from mitochondrial insult and can serve as a scaffold for the design and development of sporadic neuropathies.
Elsevier
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