About: Disrupted ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients     Goto   Sponge   NotDistinct   Permalink

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Description
  • p66Shc is an adaptor protein involved in cell proliferation and differentiation that undergoes phosphorylation at Ser36 in response to oxidative stimuli, consequently inducing a burst of reactive oxygen species (ROS), mitochondrial disruption and apoptosis. Its role during several pathologies suggests that p66Shc mitochondrial signalling can perpetuate a primary mitochondrial defect, thus contributing to the pathophysiology of that condition. Here, we show that in the fibroblasts of neuropathy, ataxia and retinitis pigmentosa (NARP) patients, the p66Shc phosphorylation pathway is significantly induced in response to intracellular oxidative stress related to disrupted ATP synthase activity and mitochondrial membrane hyperpolarisation. We postulate that the increased phosphorylation of p66Shc at Ser36 is partially responsible for further increasing ROS production, resulting in oxidative damage of proteins. Oxidative stress and p66Shc phosphorylation at Ser36 may be mitigated by antioxidant administration or the use of a p66Shc phosphorylation inhibitor
  • p66Shc is an adaptor protein involved in cell proliferation and differentiation that undergoes phosphorylation at Ser36 in response to oxidative stimuli, consequently inducing a burst of reactive oxygen species (ROS), mitochondrial disruption and apoptosis. Its role during several pathologies suggests that p66Shc mitochondrial signalling can perpetuate a primary mitochondrial defect, thus contributing to the pathophysiology of that condition. Here, we show that in the fibroblasts of neuropathy, ataxia and retinitis pigmentosa (NARP) patients, the p66Shc phosphorylation pathway is significantly induced in response to intracellular oxidative stress related to disrupted ATP synthase activity and mitochondrial membrane hyperpolarisation. We postulate that the increased phosphorylation of p66Shc at Ser36 is partially responsible for further increasing ROS production, resulting in oxidative damage of proteins. Oxidative stress and p66Shc phosphorylation at Ser36 may be mitigated by antioxidant administration or the use of a p66Shc phosphorylation inhibitor (en)
Title
  • Disrupted ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients
  • Disrupted ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients (en)
skos:prefLabel
  • Disrupted ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients
  • Disrupted ATP synthase activity and mitochondrial hyperpolarisation-dependent oxidative stress is associated with p66Shc phosphorylation in fibroblasts of NARP patients (en)
skos:notation
  • RIV/67985823:_____/13:00391778!RIV14-MSM-67985823
http://linked.open...avai/riv/aktivita
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  • I, P(GAP305/12/1247), P(MEB051040), Z(AV0Z50110509)
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  • 1
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  • 69921
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  • RIV/67985823:_____/13:00391778
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  • p66Shc; NARP; mitochondria; reactive oxygen species (en)
http://linked.open.../riv/klicoveSlovo
http://linked.open...odStatuVydavatele
  • GB - Spojené království Velké Británie a Severního Irska
http://linked.open...ontrolniKodProRIV
  • [1FB834BEB102]
http://linked.open...i/riv/nazevZdroje
  • International Journal of Biochemistry and Cell Biology
http://linked.open...in/vavai/riv/obor
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http://linked.open...v/svazekPeriodika
  • 45
http://linked.open...iv/tvurceVysledku
  • Ježek, Petr
  • Tauber, Jan
  • Diogo, C. T.
  • Duszynski, J.
  • Karkucinska-Wieckowska, A.
  • Lebiedzinska, M.
  • Oliveira, P. J.
  • Pinton, P.
  • Pronicki, M.
  • Suski, J. M.
  • Szabadkai, G.
  • Wieckowski, M. R.
  • Wilczynski, G.
  • Wlodarczyk, J.
  • Wojtala, A.
http://linked.open...ain/vavai/riv/wos
  • 000314073400021
http://linked.open...n/vavai/riv/zamer
issn
  • 1357-2725
number of pages
http://bibframe.org/vocab/doi
  • 10.1016/j.biocel.2012.07.020
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