KEGG   NETWORK: N01197
Entry
N01197                      Network                                

Name
Scrapie conformation PrPSc to 26S proteasome-mediated protein degradation
Definition
PRNP* -| 26S
  Expanded
5621v1 -| (5687+5683+5685+5688+143471+5686+5682+5684+5694+5695+5691+5690+5693+5689+5692+5701+5700+5704+5706+5702+5705+5708+5707+5709+5715+5718+5717+9861+5713+5719+5710+10213+5714+11047+7979)
Class
nt06420 Ubiquitin-proteasome system
nt06465 Prion disease
Type
Variant
Pathway
hsa05020  Prion disease
Disease
H00061  Prion disease
Gene
5621  PRNP; prion protein
5687  PSMA6; proteasome 20S subunit alpha 6
5683  PSMA2; proteasome 20S subunit alpha 2
5685  PSMA4; proteasome 20S subunit alpha 4
5688  PSMA7; proteasome 20S subunit alpha 7
143471  PSMA8; proteasome 20S subunit alpha 8
5686  PSMA5; proteasome 20S subunit alpha 5
5682  PSMA1; proteasome 20S subunit alpha 1
5684  PSMA3; proteasome 20S subunit alpha 3
5694  PSMB6; proteasome 20S subunit beta 6
5695  PSMB7; proteasome 20S subunit beta 7
5691  PSMB3; proteasome 20S subunit beta 3
5690  PSMB2; proteasome 20S subunit beta 2
5693  PSMB5; proteasome 20S subunit beta 5
5689  PSMB1; proteasome 20S subunit beta 1
5692  PSMB4; proteasome 20S subunit beta 4
5701  PSMC2; proteasome 26S subunit, ATPase 2
5700  PSMC1; proteasome 26S subunit, ATPase 1
5704  PSMC4; proteasome 26S subunit, ATPase 4
5706  PSMC6; proteasome 26S subunit, ATPase 6
5702  PSMC3; proteasome 26S subunit, ATPase 3
5705  PSMC5; proteasome 26S subunit, ATPase 5
5708  PSMD2; proteasome 26S subunit, non-ATPase 2
5707  PSMD1; proteasome 26S subunit, non-ATPase 1
5709  PSMD3; proteasome 26S subunit, non-ATPase 3
5715  PSMD9; proteasome 26S subunit, non-ATPase 9
5718  PSMD12; proteasome 26S subunit, non-ATPase 12
5717  PSMD11; proteasome 26S subunit, non-ATPase 11
9861  PSMD6; proteasome 26S subunit, non-ATPase 6
5713  PSMD7; proteasome 26S subunit, non-ATPase 7
5719  PSMD13; proteasome 26S subunit, non-ATPase 13
5710  PSMD4; proteasome 26S subunit, non-ATPase 4
10213  PSMD14; proteasome 26S subunit, non-ATPase 14
5714  PSMD8; proteasome 26S subunit, non-ATPase 8
11047  ADRM1; adhesion regulating molecule 1
7979  SEM1; SEM1 26S proteasome complex subunit
Variant
5621v1 (PRNP*)  PRNP scrapie conformation
Reference
  Authors
Kristiansen M, Deriziotis P, Dimcheff DE, Jackson GS, Ovaa H, Naumann H, Clarke AR, van Leeuwen FW, Menendez-Benito V, Dantuma NP, Portis JL, Collinge J, Tabrizi SJ
  Title
Disease-associated prion protein oligomers inhibit the 26S proteasome.
  Journal
Mol Cell 26:175-88 (2007)
DOI:10.1016/j.molcel.2007.04.001
Reference
  Authors
Andre R, Tabrizi SJ
  Title
Misfolded PrP and a novel mechanism of proteasome inhibition.
  Journal
Prion 6:32-6 (2012)
DOI:10.4161/pri.6.1.18272
Reference
  Authors
Deriziotis P, Andre R, Smith DM, Goold R, Kinghorn KJ, Kristiansen M, Nathan JA, Rosenzweig R, Krutauz D, Glickman MH, Collinge J, Goldberg AL, Tabrizi SJ
  Title
Misfolded PrP impairs the UPS by interaction with the 20S proteasome and inhibition of substrate entry.
  Journal
EMBO J 30:3065-77 (2011)
DOI:10.1038/emboj.2011.224
Reference
  Authors
Zhu T, Hayat Khan S, Zhao D, Yang L
  Title
Regulation of proteasomes in prion disease.
  Journal
Acta Biochim Biophys Sin (Shanghai) 46:531-9 (2014)
DOI:10.1093/abbs/gmu031
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