KEGG   PATHWAY: zca05020
Entry
zca05020                    Pathway                                
Name
Prion disease - Zalophus californianus (California sea lion)
Description
Prion diseases, also termed transmissible spongiform encephalopathies (TSEs), are a group of fatal neurodegenerative diseases that affect humans and a number of other animal species. The etiology of these diseases is thought to be associated with the conversion of a normal protein, PrPC, into an infectious, pathogenic form, PrPSc. The conversion is induced by prion infections (for example, variant Creutzfeldt-Jakob disease (vCJD), iatrogenic CJD, Kuru), mutations (familial CJD, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia (FFI)) or unknown factors (sporadic CJD (sCJD)), and is thought to occur after PrPC has reached the plasma membrane or is re-internalized for degradation. The PrPSc form shows greater protease resistance than PrPC and accumulates in affected individuals, often in the form of extracellular plaques. Pathways that may lead to neuronal death comprise oxidative stress, regulated activation of complement, ubiquitin-proteasome and endosomal-lysosomal systems, synaptic alterations and dendritic atrophy, corticosteroid response, and endoplasmic reticulum stress. In addition, the conformational transition could lead to the lost of a beneficial activity of the natively folded protein, PrPC.
Class
Human Diseases; Neurodegenerative disease
Pathway map
zca05020  Prion disease
zca05020

Disease
H00061  Prion disease
Organism
Zalophus californianus (California sea lion) [GN:zca]
Gene
113908025  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 isoform X1 [KO:K03033]
113908037  [KO:K03945]
113908104  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
113908589  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
113908627  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
113908731  [KO:K00235] [EC:1.3.5.1]
113908736  RYR3; ryanodine receptor 3 [KO:K04963]
113908853  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
113908866  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
113908893  [KO:K02272]
113908954  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
113909121  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
113909155  [KO:K03948]
113909577  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
113909591  CASP9; LOW QUALITY PROTEIN: caspase-9 [KO:K04399] [EC:3.4.22.62]
113909655  [KO:K03945]
113909911  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
113909952  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
113910035  [KO:K02264]
113910090  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
113910274  [KO:K02137]
113910494  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
113910511  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
113910552  [KO:K02131]
113910988  [KO:K02268]
113911031  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
113911261  [KO:K08738]
113911293  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
113911305  [KO:K02137]
113911381  [KO:K03948]
113911411  [KO:K02266]
113911464  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
113911563  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
113911583  [KO:K03947]
113911619  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
113911645  [KO:K03937]
113911688  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
113911693  CAV1; caveolin-1 isoform X2 [KO:K06278]
113911734  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
113911785  [KO:K02128]
113911795  [KO:K03957]
113911872  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
113911890  CAV2; caveolin-2 [KO:K12958]
113911895  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
113911910  IL6; interleukin-6 [KO:K05405]
113912320  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
113912409  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
113912413  [KO:K02128]
113912479  [KO:K03957]
113912583  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
113912591  [KO:K02127]
113912698  [KO:K02266]
113912785  [KO:K02266]
113912908  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
113912962  [KO:K00419]
113913086  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
113913107  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
113913731  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
113913831  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
113913901  [KO:K02272]
113913911  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
113913999  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
113914078  [KO:K03945]
113914094  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
113914243  [KO:K02271]
113914325  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
113914353  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
113914357  [KO:K03947]
113914501  [KO:K02273]
113914539  BAD; bcl2-associated agonist of cell death [KO:K02158]
113914678  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
113914679  [KO:K02270]
113914728  [KO:K00411] [EC:7.1.1.8]
113914779  [KO:K03943] [EC:7.1.1.2]
113914893  [KO:K03947]
113915003  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
113915048  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
113915131  [KO:K02271]
113915204  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
113915210  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
113915225  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
113915330  [KO:K00417]
113915657  [KO:K03948]
113915754  C8B; complement component C8 beta chain isoform X1 [KO:K03998]
113915839  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
113915905  [KO:K00236]
113916151  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
113916205  [KO:K03957]
113916266  C1QB; complement C1q subcomponent subunit B [KO:K03987]
113916286  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
113916473  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
113916546  [KO:K02128]
113916636  TUBB4A; tubulin beta-4A chain [KO:K07375]
113916653  [KO:K00420]
113916664  [KO:K02136]
113916725  [KO:K00417]
113916759  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
113916795  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X2 [KO:K04851]
113916866  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
113917002  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
113917022  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
113917088  ATP5F1D; LOW QUALITY PROTEIN: ATP synthase subunit delta, mitochondrial [KO:K02134]
113917179  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
113917199  [KO:K03945]
113917259  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
113917309  [KO:K00414]
113917422  GRIN3B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 3B [KO:K05214]
113917543  CAV3; caveolin-3 [KO:K12959]
113917598  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
113917812  [KO:K02272]
113917862  [KO:K03945]
113917893  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
113917957  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
113917990  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
113918159  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
113918169  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
113918208  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
113918479  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
113918509  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
113918529  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
113918544  ATP5PO; ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
113918704  [KO:K03948]
113918892  [KO:K03945]
113918898  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
113918932  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
113919173  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
113919175  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
113919323  RYR2; LOW QUALITY PROTEIN: ryanodine receptor 2 [KO:K04962]
113919327  [KO:K03946]
113919467  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
113919493  [KO:K02267]
113919503  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
113919563  [KO:K02271]
113919632  KIF5C; kinesin heavy chain [KO:K10396]
113919696  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
113919721  [KO:K02138]
113919925  [KO:K03949]
113919926  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
113919936  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
113919987  [KO:K04392]
113920123  [KO:K00420]
113920393  [KO:K03943] [EC:7.1.1.2]
113920563  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
113920580  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
113920583  [KO:K03945]
113920673  [KO:K02138]
113920690  [KO:K03945]
113920876  [KO:K07374]
113920877  [KO:K02267]
113920996  [KO:K00417]
113921409  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
113921493  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
113921503  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
113921591  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
113921668  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
113921728  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
113921757  CACNA1C; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
113921771  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
113921855  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
113921886  [KO:K02138]
113921922  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
113921975  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
113921994  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
113922046  [KO:K15041]
113922064  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
113922087  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
113922091  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
113922330  [KO:K07374]
113922331  [KO:K07374]
113922403  [KO:K02270]
113922573  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
113922602  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
113922696  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
113922724  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
113922735  [KO:K02268]
113922775  [KO:K02138]
113922790  [KO:K03947]
113922818  [KO:K03945]
113922861  [KO:K02267]
113922885  [KO:K02266]
113922888  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
113922904  [KO:K00417]
113923107  NCF4; neutrophil cytosol factor 4 [KO:K08012]
113923113  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
113923387  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
113923487  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
113923573  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
113923759  [KO:K02263]
113923771  [KO:K02273]
113923802  [KO:K03949]
113923899  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
113924059  [KO:K03967]
113924066  [KO:K02273]
113924138  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
113924279  [KO:K02138]
113924334  [KO:K02131]
113924373  [KO:K02265]
113924414  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
113924501  [KO:K05863]
113924509  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
113924510  [KO:K02128]
113924761  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
113924798  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
113924817  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
113924844  [KO:K02272]
113924949  [KO:K00416]
113924993  [KO:K02127]
113925088  [KO:K02271]
113925168  [KO:K02131]
113925227  [KO:K02270]
113925439  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial isoform X1 [KO:K03967]
113925460  [KO:K03115]
113925488  [KO:K03283]
113925640  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
113925679  [KO:K03957]
113925738  [KO:K00235] [EC:1.3.5.1]
113925863  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
113925955  SLC25A31; ADP/ATP translocase 4 isoform X1 [KO:K05863]
113926255  [KO:K10881]
113926300  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
113926313  [KO:K00413]
113926549  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
113926974  [KO:K08738]
113926987  C1QA; complement C1q subcomponent subunit A [KO:K03986]
113927034  [KO:K05862]
113927035  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
113927124  [KO:K07375]
113927125  TUBB2A; tubulin beta-2A chain isoform X1 [KO:K07375]
113927173  [KO:K04565] [EC:1.15.1.1]
113927199  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
113927231  [KO:K00237]
113927239  [KO:K03949]
113927275  [KO:K02730] [EC:3.4.25.1]
113927342  [KO:K03115]
113927359  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
113927447  PSMB1; proteasome subunit beta type-1 isoform X1 [KO:K02732] [EC:3.4.25.1]
113927499  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
113927572  [KO:K02273]
113927616  [KO:K02270]
113927727  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
113927729  HSPA1B; heat shock 70 kDa protein 1B [KO:K03283]
113927740  TNF; tumor necrosis factor [KO:K03156]
113927749  CSNK2B; casein kinase II subunit beta isoform X1 [KO:K03115]
113927778  [KO:K03938]
113927801  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
113927996  TUBB; tubulin beta chain isoform X1 [KO:K07375]
113928413  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
113928501  [KO:K03944]
113928528  [KO:K03283]
113928769  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
113928810  C7; complement component C7 isoform X1 [KO:K03996]
113928873  [KO:K02267]
113928897  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
113928925  [KO:K03949]
113928933  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
113928989  [KO:K00418]
113929025  [KO:K03097] [EC:2.7.11.1]
113929082  C6; complement component C6 [KO:K03995]
113929103  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
113929105  [KO:K02138]
113929199  [KO:K02128]
113929477  [KO:K00417]
113929521  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
113929525  C9; complement component C9 isoform X1 [KO:K04000]
113929533  [KO:K03945]
113929574  EGR1; early growth response protein 1 [KO:K09203]
113929658  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
113929839  [KO:K02127]
113929849  [KO:K02272]
113929909  [KO:K00417]
113930220  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
113930268  [KO:K21421] [EC:1.-.-.-]
113930576  [KO:K03283]
113930718  [KO:K02263]
113930829  [KO:K02138]
113931031  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
113931143  [KO:K07374]
113931189  [KO:K03937]
113931221  [KO:K03035]
113931264  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
113931319  [KO:K03947]
113931398  [KO:K03948]
113931401  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
113931446  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
113931478  [KO:K02138]
113931482  [KO:K02272]
113931503  [KO:K02271]
113931520  [KO:K02136]
113931739  NCF1; neutrophil cytosol factor 1 [KO:K08011]
113931821  PRKACB; LOW QUALITY PROTEIN: cAMP-dependent protein kinase catalytic subunit beta [KO:K04345] [EC:2.7.11.11]
113931998  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
113932092  LAMC1; laminin subunit gamma-1 isoform X1 [KO:K05635]
113932138  [KO:K02266]
113932201  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
113932312  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
113932378  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
113932406  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
113932454  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
113932530  [KO:K00415]
113932787  [KO:K02267]
113932824  [KO:K03115]
113932864  [KO:K07374]
113932917  [KO:K02131]
113932958  [KO:K02273]
113933001  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
113933086  [KO:K02131]
113933111  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
113933258  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
113933268  [KO:K05862]
113933286  [KO:K02266]
113933553  [KO:K05863]
113933567  [KO:K03948]
113933690  [KO:K02267]
113933699  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
113933996  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
113934022  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
113934048  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
113934203  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
113934315  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
113934570  NOTCH1; neurogenic locus notch homolog protein 1 isoform X1 [KO:K02599]
113934578  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
113934604  TUBB4B; tubulin beta-4B chain [KO:K07375]
113934630  C8G; complement component C8 gamma chain [KO:K03999]
113934784  [KO:K03945]
113934839  [KO:K02131]
113934907  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
113934908  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
113934947  C5; complement C5 [KO:K03994]
113935070  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
113935276  [KO:K07374]
113935471  [KO:K07375]
113935520  RYR1; LOW QUALITY PROTEIN: ryanodine receptor 1 [KO:K04961]
113935658  [KO:K02267]
113935661  [KO:K02270]
113935677  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
113935893  [KO:K02267]
113935895  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
113935939  [KO:K02263]
113935942  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
113936090  [KO:K00411] [EC:7.1.1.8]
113936123  [KO:K02267]
113936225  [KO:K02131]
113936426  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
113936546  KLC3; kinesin light chain 3 isoform X2 [KO:K10407]
113936564  [KO:K08009]
113936636  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
113936647  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
113936650  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
113936745  [KO:K02270]
113936755  [KO:K02128]
113936867  [KO:K07374]
113936929  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
113936988  [KO:K02263]
113937005  [KO:K03938]
113937056  CSNK2A1; LOW QUALITY PROTEIN: casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
113937102  [KO:K02138]
113937313  [KO:K02266]
113937385  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
113937435  [KO:K03945]
113937504  [KO:K03948]
113937506  [KO:K02265]
113937551  [KO:K02725] [EC:3.4.25.1]
113937669  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
113937717  [KO:K02128]
113937839  IL1B; interleukin-1 beta [KO:K04519]
113937908  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
113938009  [KO:K02272]
113938051  [KO:K03948]
113938055  [KO:K05634]
113938273  IL1A; interleukin-1 alpha isoform X1 [KO:K04383]
113938347  [KO:K02270]
113938352  [KO:K03948]
113938488  [KO:K07374]
113938523  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
113938644  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
113938649  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
113938694  [KO:K02273]
113939093  CCL5; C-C motif chemokine 5 [KO:K12499]
113939295  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
113939471  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
113939515  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
113939560  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
113939602  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
113939696  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
113939837  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
113939860  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
118355935  [KO:K04741] [EC:3.4.22.-]
118355947  C1QC; complement C1q subcomponent subunit C [KO:K03988]
118356054  [KO:K02270]
118356158  [KO:K07374]
118356194  [KO:K02270]
118356237  [KO:K07374]
118356266  [KO:K07374]
118356289  [KO:K02138]
118356418  [KO:K02138]
118356633  [KO:K03945]
118356768  [KO:K02726] [EC:3.4.25.1]
118356769  [KO:K02268]
118356796  [KO:K03965]
118356834  [KO:K02127]
118356882  [KO:K03945]
118356894  [KO:K02271]
118356917  [KO:K03960]
118357011  [KO:K02138]
118357105  [KO:K02138]
118357183  [KO:K03283]
118357243  [KO:K02138]
118357304  [KO:K07374]
118357322  [KO:K07374]
4355788  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
4355789  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
4355790  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
4355791  COX2; cytochrome c oxidase subunit II [KO:K02261]
4355792  COX3; cytochrome c oxidase subunit III [KO:K02262]
4355793  CYTB; cytochrome b [KO:K00412]
4355794  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
4355795  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
4355796  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
4355797  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
4355798  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
4355799  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
4355800  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
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Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
DOI:10.1017/erm.2016.8
Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
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Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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Soto C, Satani N
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The intricate mechanisms of neurodegeneration in prion diseases.
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Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
PMID:27060771
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Mays CE, Soto C
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The stress of prion disease.
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Brain Res 1648:553-560 (2016)
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Reference
PMID:32565065
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Genetic risk factors for Creutzfeldt-Jakob disease.
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Neurobiol Dis 142:104973 (2020)
DOI:10.1016/j.nbd.2020.104973
Reference
PMID:18245809
  Authors
Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
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Am J Pathol 172:555-65 (2008)
DOI:10.2353/ajpath.2008.070442
Reference
PMID:15695097
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
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Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
PMID:17051207
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Caughey B, Baron GS
  Title
Prions and their partners in crime.
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Nature 443:803-10 (2006)
DOI:10.1038/nature05294
Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
PMID:17085779
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Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
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J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
PMID:14705136
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Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
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J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
PMID:10398674
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
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Reference
PMID:15890824
  Authors
Novakofski J, Brewer MS, Mateus-Pinilla N, Killefer J, McCusker RH
  Title
Prion biology relevant to bovine spongiform encephalopathy.
  Journal
J Anim Sci 83:1455-76 (2005)
DOI:10.2527/2005.8361455x
Reference
PMID:27581199
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
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Biochem Biophys Res Commun 483:1148-1155 (2017)
DOI:10.1016/j.bbrc.2016.07.118
Reference
PMID:31618844
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
  Journal
Int J Mol Sci 20:E5107 (2019)
DOI:10.3390/ijms20205107
Reference
PMID:31795242
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
  Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
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Int J Mol Sci 20:E6004 (2019)
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Reference
PMID:30710214
  Authors
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
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Mol Neurobiol 56:6035-6045 (2019)
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Reference
PMID:23439666
  Authors
Song Z, Zhao D, Yang L
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Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
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Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
PMID:28643372
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
PMID:29194372
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
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Pathogens 6:63 (2017)
DOI:10.3390/pathogens6040063
Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
PMID:29476642
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
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FEBS J 286:342-355 (2019)
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Reference
PMID:21722302
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Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
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Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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J Cell Mol Med 15:2025-39 (2011)
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Reference
PMID:18420416
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
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Neurobiol Dis 30:331-42 (2008)
DOI:10.1016/j.nbd.2008.02.003
Reference
PMID:16844381
  Authors
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
  Title
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
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Neurobiol Dis 23:669-78 (2006)
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Reference
PMID:21980554
  Authors
Torres M, Encina G, Soto C, Hetz C
  Title
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
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Commun Integr Biol 4:258-61 (2011)
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Reference
PMID:25766616
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Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
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Exp Mol Med 47:e147 (2015)
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Reference
PMID:17466621
  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
PMID:10195903
  Authors
Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
  Title
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Science 284:339-43 (1999)
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Reference
PMID:14532116
  Authors
Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
  Title
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
  Journal
EMBO J 22:5435-45 (2003)
DOI:10.1093/emboj/cdg537
Reference
PMID:31417367
  Authors
Zamponi E, Pigino GF
  Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
  Journal
Front Cell Neurosci 13:350 (2019)
DOI:10.3389/fncel.2019.00350
Reference
PMID:29261664
  Authors
Zamponi E, Buratti F, Cataldi G, Caicedo HH, Song Y, Jungbauer LM, LaDu MJ, Bisbal M, Lorenzo A, Ma J, Helguera PR, Morfini GA, Brady ST, Pigino GF
  Title
Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
  Journal
PLoS One 12:e0188340 (2017)
DOI:10.1371/journal.pone.0188340
Reference
PMID:21196165
  Authors
Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
  Title
Understanding the neurospecificity of Prion protein signaling.
  Journal
Front Biosci (Landmark Ed) 16:169-86 (2011)
DOI:10.2741/3682
Reference
PMID:23479001
  Authors
Didonna A
  Title
Prion protein and its role in signal transduction.
  Journal
Cell Mol Biol Lett 18:209-30 (2013)
DOI:10.2478/s11658-013-0085-0
Reference
PMID:28507517
  Authors
Shah SZA, Zhao D, Hussain T, Yang L
  Title
The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
  Journal
Front Aging Neurosci 9:120 (2017)
DOI:10.3389/fnagi.2017.00120
Reference
PMID:15528202
  Authors
Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
  Title
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
  Journal
J Biol Chem 280:1529-34 (2005)
DOI:10.1074/jbc.M410966200
Reference
PMID:15640354
  Authors
Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
  Title
Notch-1 activation and dendritic atrophy in prion disease.
  Journal
Proc Natl Acad Sci U S A 102:886-91 (2005)
DOI:10.1073/pnas.0408612101
Reference
PMID:20205843
  Authors
Dearmond SJ, Bajsarowicz K
  Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
  Journal
Mol Neurodegener 5:6 (2010)
DOI:10.1186/1750-1326-5-6
Reference
PMID:29998397
  Authors
Hirsch TZ, Martin-Lanneree S, Reine F, Hernandez-Rapp J, Herzog L, Dron M, Privat N, Passet B, Halliez S, Villa-Diaz A, Lacroux C, Klein V, Haik S, Andreoletti O, Torres JM, Vilotte JL, Beringue V, Mouillet-Richard S
  Title
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases.
  Journal
Mol Neurobiol 56:2159-2173 (2019)
DOI:10.1007/s12035-018-1193-7
Reference
PMID:28714865
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
PMID:15342004
  Authors
Mabbott NA
  Title
The complement system in prion diseases.
  Journal
Curr Opin Immunol 16:587-93 (2004)
DOI:10.1016/j.coi.2004.07.002
Reference
PMID:15611724
  Authors
Mallucci G, Collinge J
  Title
Rational targeting for prion therapeutics.
  Journal
Nat Rev Neurosci 6:23-34 (2005)
DOI:10.1038/nrn1584
Related
pathway
zca00190  Oxidative phosphorylation
zca03050  Proteasome
zca04020  Calcium signaling pathway
zca04140  Autophagy - animal
zca04141  Protein processing in endoplasmic reticulum
zca04142  Lysosome
zca04144  Endocytosis
zca04210  Apoptosis
zca04330  Notch signaling pathway
zca04610  Complement and coagulation cascades
zca04668  TNF signaling pathway
KO pathway
ko05020   

DBGET integrated database retrieval system