PMID- 30337853
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20200929
IS  - 1662-5099 (Print)
IS  - 1662-5099 (Electronic)
IS  - 1662-5099 (Linking)
VI  - 11
DP  - 2018
TI  - p62-Keap1-NRF2-ARE Pathway: A Contentious Player for Selective Targeting of 
      Autophagy, Oxidative Stress and Mitochondrial Dysfunction in Prion Diseases.
PG  - 310
LID - 10.3389/fnmol.2018.00310 [doi]
LID - 310
AB  - Prion diseases are a group of fatal and debilitating neurodegenerative diseases 
      affecting humans and animal species. The conversion of a non-pathogenic normal 
      cellular protein (PrP(c)) into an abnormal infectious, protease-resistant, 
      pathogenic form prion protein scrapie (PrP(Sc)), is considered the etiology of 
      these diseases. PrP(Sc) accumulates in the affected individual's brain in the 
      form of extracellular plaques. The molecular pathways leading to neuronal cell 
      death in prion diseases are still unclear. The free radical damage, oxidative 
      stress and mitochondrial dysfunction play a key role in the pathogenesis of the 
      various neurodegenerative disorders including prion diseases. The brain is very 
      sensitive to changes in the redox status. It has been demonstrated that PrP(c) 
      behaves as an antioxidant, while the neurotoxic prion peptide PrP(Sc) increases 
      hydrogen peroxide toxicity in the neuronal cultures leading to mitochondrial 
      dysfunction and cell death. The nuclear factor erythroid 2-related factor 2 
      (NRF2) is an oxidative responsive pathway and a guardian of lifespan, which 
      protect the cells from free radical stress-mediated cell death. The reduced 
      glutathione, a major small molecule antioxidant present in all mammalian cells, 
      and produced by several downstream target genes of NRF2, counterbalances the 
      mitochondrial reactive oxygen species (ROS) production. In recent years, it has 
      emerged that the ubiquitin-binding protein, p62-mediated induction of autophagy, 
      is crucial for NRF2 activation and elimination of mitochondrial dysfunction and 
      oxidative stress. The current review article, focuses on the role of NRF2 pathway 
      in prion diseases to mitigate the disease progression.
FAU - Shah, Syed Zahid Ali
AU  - Shah SZA
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
FAU - Zhao, Deming
AU  - Zhao D
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
FAU - Hussain, Tariq
AU  - Hussain T
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
FAU - Sabir, Naveed
AU  - Sabir N
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
FAU - Mangi, Mazhar Hussain
AU  - Mangi MH
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
FAU - Yang, Lifeng
AU  - Yang L
AD  - National Animal Transmissible Spongiform Encephalopathy Laboratory, Key 
      Laboratory of Animal Epidemiology and Zoonosis of Ministry of Agriculture, 
      College of Veterinary Medicine and State Key Laboratory of Agrobiotechnology, 
      China Agricultural University, Beijing, China.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20181004
PL  - Switzerland
TA  - Front Mol Neurosci
JT  - Frontiers in molecular neuroscience
JID - 101477914
PMC - PMC6180192
OTO - NOTNLM
OT  - extracellular plaques
OT  - hydrogen peroxide toxicity
OT  - mitochondrial dysfunction
OT  - nuclear factor erythroid 2-related factor 2 (NRF2)
OT  - prion protein scrapie (PrPSc)
EDAT- 2018/10/20 06:00
MHDA- 2018/10/20 06:01
PMCR- 2018/01/01
CRDT- 2018/10/20 06:00
PHST- 2018/05/15 00:00 [received]
PHST- 2018/08/14 00:00 [accepted]
PHST- 2018/10/20 06:00 [entrez]
PHST- 2018/10/20 06:00 [pubmed]
PHST- 2018/10/20 06:01 [medline]
PHST- 2018/01/01 00:00 [pmc-release]
AID - 10.3389/fnmol.2018.00310 [doi]
PST - epublish
SO  - Front Mol Neurosci. 2018 Oct 4;11:310. doi: 10.3389/fnmol.2018.00310. eCollection 
      2018.