KEGG   PATHWAY: eju05020
Entry
eju05020                    Pathway                                
Name
Prion disease - Eumetopias jubatus (Steller 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
eju05020  Prion disease
eju05020

Organism
Eumetopias jubatus (Steller sea lion) [GN:eju]
Gene
114220784  PRNP; major prion protein [KO:K05634]
114218666  NCAM2; neural cell adhesion molecule 2 [KO:K06491]
114211434  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
114222807  LAMC1; laminin subunit gamma-1 [KO:K05635]
114217149  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
114220383  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
114208397  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
114197723  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
114199627  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
114209890  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
114201744  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
114219938  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
114221701  GRIN2B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2B [KO:K05210]
114216269  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
114197198  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
114213551  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
114226323  GRIN3B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 3B [KO:K05214]
114201734  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
114222521  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
114225091  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
114216787  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
114221937  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
114212793  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
114223436  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
114221283  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
114218458  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
114215099  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
114198938  RYR1; ryanodine receptor 1 [KO:K04961]
114210255  RYR2; ryanodine receptor 2 [KO:K04962]
114221310  RYR3; ryanodine receptor 3 [KO:K04963]
114207665  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
114216613  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
114222155  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
114211415  caspase-12-like [KO:K04741] [EC:3.4.22.-]
114217489  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
114201320  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
114214123  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
114218910  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
114213552  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
114220328  BAD; bcl2-associated agonist of cell death [KO:K02158]
114209486  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
114214136  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
114224842  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
114208278  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
114218042  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
114196999  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
114206185  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
114214213  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
114222925  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
114224279  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
114221249  cytochrome c [KO:K08738]
114213333  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
114206181  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
114214165  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
805015  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
805011  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
805018  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
805010  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
805012  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
805020  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
805019  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
114220454  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
114224701  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
114207265  NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial-like [KO:K03943] [EC:7.1.1.2]
114226430  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
114225274  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
114197002  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
114207684  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
114209459  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
114215909  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
114201803  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
114214196  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
114223537  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
114209740  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
114222308  NDUFA4; cytochrome c oxidase subunit NDUFA4 isoform X1 [KO:K03948]
114215089  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
114209382  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
114210700  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
114205498  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
114222773  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
114218373  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
114200879  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
114212844  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
114222562  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
114210798  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
114198808  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
114226302  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
114210939  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
114196450  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
114208394  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
114206853  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
114211582  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
114225925  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
114214703  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
114206935  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
114218163  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
114197646  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
114205347  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
114211191  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
114215680  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
114216838  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
114215976  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
114199119  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
114215277  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
114211827  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
114224147  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
114198559  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
114226204  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
114220293  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
114208220  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
114218791  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
114198554  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
114213172  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
114221094  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
114199177  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
114211322  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
114224452  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
805017  CYTB; cytochrome b [KO:K00412]
114201119  cytochrome c1, heme protein, mitochondrial [KO:K00413]
114225028  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
114198773  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
114224286  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
114216460  cytochrome b-c1 complex subunit 7 [KO:K00417]
114214091  cytochrome b-c1 complex subunit 7 [KO:K00417]
114209508  cytochrome b-c1 complex subunit 8 [KO:K00418]
114224572  cytochrome b-c1 complex subunit 10 [KO:K00420]
114226218  cytochrome b-c1 complex subunit 10 [KO:K00420]
805021  COX3; cytochrome c oxidase subunit III [KO:K02262]
805016  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
805022  COX2; cytochrome c oxidase subunit II [KO:K02261]
114203191  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
114220843  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
114206521  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
114209841  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
114219053  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
114214523  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
114224416  cytochrome c oxidase subunit 6B1 [KO:K02267]
114202036  cytochrome c oxidase subunit 6B2 [KO:K02267]
114211303  cytochrome c oxidase subunit 6B1-like [KO:K02267]
114202808  cytochrome c oxidase subunit 6B1-like [KO:K02267]
114212897  cytochrome c oxidase subunit 6B1-like [KO:K02267]
114220977  cytochrome c oxidase subunit 6B1-like [KO:K02267]
114213397  cytochrome c oxidase subunit 6C [KO:K02268]
114197981  cytochrome c oxidase subunit 6C-like [KO:K02268]
114200962  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
114219688  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
114204451  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
114200424  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
114210260  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
114207401  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
114209323  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
114217665  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
114220164  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
114205171  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
114214435  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
114222778  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
114223423  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
114204960  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
114214966  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
114217707  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
114202511  ATP synthase subunit gamma, mitochondrial-like [KO:K02136]
114212104  ATP synthase subunit gamma, mitochondrial-like [KO:K02136]
114226202  ATP5F1D; LOW QUALITY PROTEIN: ATP synthase subunit delta, mitochondrial [KO:K02134]
114197956  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
114199134  LOW QUALITY PROTEIN: ATP synthase subunit epsilon-like protein, mitochondrial [KO:K02135]
805013  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
114207939  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
114201181  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
114217200  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
114225617  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
114225857  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
114202289  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
114207119  ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
114216159  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
114208852  ATP synthase subunit d, mitochondrial [KO:K02138]
114209232  ATP synthase subunit d, mitochondrial-like [KO:K02138]
114218932  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
114200800  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114217478  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114218238  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
114218266  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114219871  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114203795  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114204217  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114204225  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114204234  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114206127  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
114215493  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
805014  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
114217899  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
114213690  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
114201261  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
114205097  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
114199283  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
114207883  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
114219412  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
114216240  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
114225451  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
114212763  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
114198481  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
114217117  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
114217946  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
114209318  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
114197353  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
114221645  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
114204967  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
114198990  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
114214186  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
114215393  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
114196704  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
114207019  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
114210830  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
114198465  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 isoform X1 [KO:K03033]
114219152  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
114196748  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
114213893  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
114214654  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
114203515  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
114201522  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
114197261  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
114216518  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
114198959  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
114199280  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
114220907  26S proteasome complex subunit SEM1 [KO:K10881]
114212820  C5; complement C5 [KO:K03994]
114204832  C6; complement component C6 [KO:K03995]
114204794  C7; complement component C7 isoform X1 [KO:K03996]
114212130  C8A; complement component C8 alpha chain [KO:K03997]
114212140  C8B; complement component C8 beta chain [KO:K03998]
114201774  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
114204828  C9; complement component C9 [KO:K04000]
114199063  C1QC; complement C1q subcomponent subunit C [KO:K03988]
114225993  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
114204297  heat shock 70 kDa protein 1 [KO:K03283]
114213501  heat shock cognate 71 kDa protein-like [KO:K03283]
114222238  heat shock 70 kDa protein 1-like [KO:K03283]
114207061  heat shock cognate 71 kDa protein-like [KO:K03283]
114201903  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
114210649  CAV1; caveolin-1 [KO:K06278]
114210654  CAV2; caveolin-2 [KO:K12958]
114207761  CAV3; caveolin-3 [KO:K12959]
114213130  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
114205617  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
114206954  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
114224308  phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649]
114224631  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
114226659  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
114225007  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
114216715  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
114200838  cytochrome b-245 light chain [KO:K08009]
114218355  NCF4; neutrophil cytosol factor 4 [KO:K08012]
114214363  NCF1; neutrophil cytosol factor 1 [KO:K08011]
114222825  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
114210095  LOW QUALITY PROTEIN: ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
114203962  ras-related C3 botulinum toxin substrate 1 [KO:K04392]
114197462  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
114218556  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
114218424  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
114218476  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
114222054  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
114222247  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
114224898  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
114201201  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
114205461  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
114202353  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
114214409  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
114215964  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
114224124  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
114218277  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
114226347  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
114211711  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
114198708  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
114215177  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
114221152  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
114203954  cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
114203955  cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
114222060  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
114209509  EGR1; early growth response protein 1 [KO:K09203]
114213872  CCL5; C-C motif chemokine 5 [KO:K12499]
114223488  IL1A; interleukin-1 alpha [KO:K04383]
114223431  IL1B; interleukin-1 beta [KO:K04519]
114221211  IL6; interleukin-6 isoform X1 [KO:K05405]
114222242  TNF; tumor necrosis factor [KO:K03156]
114214730  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
114208559  casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
114201602  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
114220654  CSNK2A1; LOW QUALITY PROTEIN: casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
114222207  CSNK2B; casein kinase II subunit beta [KO:K03115]
114216469  KIF5C; kinesin heavy chain [KO:K10396]
114222403  KIF5B; kinesin-1 heavy chain [KO:K10396]
114215062  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
114220356  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
114204461  KLC4; kinesin light chain 4 [KO:K10407]
114197165  KLC3; kinesin light chain 3 [KO:K10407]
114206413  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
114200150  tubulin alpha-4A chain isoform X1 [KO:K07374]
114208888  tubulin alpha-1B chain-like [KO:K07374]
114216837  tubulin alpha-1C chain-like [KO:K07374]
114217714  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
114217773  tubulin alpha-1A chain-like [KO:K07374]
114225742  tubulin alpha-1B chain [KO:K07374]
114225882  TUBA1A; tubulin alpha-1A chain [KO:K07374]
114225883  TUBA1C; tubulin alpha-1C chain [KO:K07374]
114201953  tubulin alpha-3 chain-like [KO:K07374]
114201963  tubulin alpha-3 chain [KO:K07374]
114218399  tubulin alpha-1A chain-like [KO:K07374]
114204266  tubulin alpha-3 chain-like [KO:K07374]
114222003  tubulin alpha-8 chain [KO:K07374]
114222582  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
114200338  tubulin beta-3 chain [KO:K07375]
114209729  tubulin beta chain [KO:K07375]
114201738  TUBB4B; tubulin beta-4B chain [KO:K07375]
114226326  TUBB4A; tubulin beta-4A chain [KO:K07375]
114197933  TUBB1; tubulin beta-1 chain [KO:K07375]
114198063  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
114199266  TUBB; tubulin beta chain [KO:K07375]
114223868  tubulin beta-2A chain [KO:K07375]
114223871  TUBB2B; tubulin beta-2B chain [KO:K07375]
114223906  tubulin beta-4B chain-like [KO:K07375]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
  Authors
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
  Authors
Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
  Journal
Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
Reference
PMID:20889378
  Authors
Soto C, Satani N
  Title
The intricate mechanisms of neurodegeneration in prion diseases.
  Journal
Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
PMID:27060771
  Authors
Mays CE, Soto C
  Title
The stress of prion disease.
  Journal
Brain Res 1648:553-560 (2016)
DOI:10.1016/j.brainres.2016.04.009
Reference
PMID:32565065
  Authors
Jones E, Mead S
  Title
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.
  Journal
Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
PMID:17051207
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
  Journal
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?
  Journal
Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
PMID:17085779
  Authors
Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
  Journal
J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
PMID:14705136
  Authors
Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
  Journal
J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
PMID:10398674
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
  Journal
Clin Microbiol Rev 12:429-44 (1999)
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.
  Journal
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.
  Journal
Int J Mol Sci 20:E6004 (2019)
DOI:10.3390/ijms20236004
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.
  Journal
Mol Neurobiol 56:6035-6045 (2019)
DOI:10.1007/s12035-019-1505-6
Reference
PMID:23439666
  Authors
Song Z, Zhao D, Yang L
  Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
  Journal
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?
  Journal
Pathogens 6:E63 (2017)
DOI:10.3390/pathogens6040063
Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
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.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
PMID:21722302
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
  Journal
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
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.
  Journal
Neurobiol Dis 23:669-78 (2006)
DOI:10.1016/j.nbd.2006.05.011
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.
  Journal
Commun Integr Biol 4:258-61 (2011)
DOI:10.4161/cib.4.3.15019
Reference
PMID:25766616
  Authors
Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
  Journal
Exp Mol Med 47:e147 (2015)
DOI:10.1038/emm.2014.117
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.
  Journal
Science 284:339-43 (1999)
DOI:10.1126/science.284.5412.339
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
eju00190  Oxidative phosphorylation
eju03050  Proteasome
eju04020  Calcium signaling pathway
eju04140  Autophagy - animal
eju04141  Protein processing in endoplasmic reticulum
eju04142  Lysosome
eju04144  Endocytosis
eju04210  Apoptosis
eju04330  Notch signaling pathway
eju04610  Complement and coagulation cascades
eju04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system