KEGG   PATHWAY: aamp05020
Entry
aamp05020                   Pathway                                
Name
Prion disease - Arvicola amphibius (Eurasian water vole)
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
aamp05020  Prion disease
aamp05020

Organism
Arvicola amphibius (Eurasian water vole) [GN:aamp]
Gene
119814701  major prion protein [KO:K05634]
119809377  Ncam1; neural cell adhesion molecule 1 [KO:K06491]
119824836  Ncam2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
119827740  Lamc1; laminin subunit gamma-1 [KO:K05635]
119825355  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
119819330  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
119825948  Stip1; stress-induced-phosphoprotein 1 [KO:K09553]
119800975  Prkacb; LOW QUALITY PROTEIN: cAMP-dependent protein kinase catalytic subunit beta [KO:K04345] [EC:2.7.11.11]
119802004  Prkaca; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
119802627  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
119821217  Fyn; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
119818834  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
119812911  Grin2a; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
119806515  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
119812477  Grin2c; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
119802465  Grin2d; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
119826595  Grin3a; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
119826753  Grin3b; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
119818472  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
119806908  Cacna1c; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
119828004  Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
119804579  Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
119827234  Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857]
119818639  Hspa5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
119808167  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
119818610  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
119822546  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
119803285  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
119820756  Ryr1; ryanodine receptor 1 isoform X1 [KO:K04961]
119817089  Ryr2; ryanodine receptor 2 [KO:K04962]
119815223  Ryr3; ryanodine receptor 3 [KO:K04963]
119806390  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
119806213  Itpr2; LOW QUALITY PROTEIN: inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
119823109  Itpr3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
119800939  Ppp3ca; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
119800012  Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
119800123  Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
119823004  Ppp3r1; calcineurin subunit B type 1 [KO:K06268]
119826596  Ppp3r2; calcineurin subunit B type 2 [KO:K06268]
119820525  Bad; bcl2-associated agonist of cell death isoform X1 [KO:K02158]
119812709  Vdac1; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1 [KO:K05862]
119800149  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
119812859  Vdac3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
119807859  ADP/ATP translocase 2-like [KO:K05863]
119800309  ADP/ATP translocase 2-like [KO:K05863]
119815168  ADP/ATP translocase 2-like [KO:K05863]
119817121  LOW QUALITY PROTEIN: ADP/ATP translocase 1-like [KO:K05863]
119804458  ADP/ATP translocase 2-like [KO:K05863]
119804649  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
119812148  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
119812681  ADP/ATP translocase 2-like [KO:K05863]
119819282  ADP/ATP translocase 2-like [KO:K05863]
119826727  Slc25a31; LOW QUALITY PROTEIN: ADP/ATP translocase 4 [KO:K05863]
119827941  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
119803346  Apaf1; apoptotic protease-activating factor 1 [KO:K02084]
119807948  cytochrome c, somatic-like [KO:K08738]
119827640  cytochrome c, somatic-like [KO:K08738]
119812378  cytochrome c, somatic [KO:K08738]
119812503  cytochrome c, somatic-like [KO:K08738]
119819008  cytochrome c, testis-specific [KO:K08738]
119807481  cytochrome c, somatic [KO:K08738]
119817619  Casp9; caspase-9 [KO:K04399] [EC:3.4.22.62]
119812861  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
119823855  Mcu; calcium uniporter protein, mitochondrial [KO:K20858]
119815378  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
119821089  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
119823492  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
119804764  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
119814379  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
119808469  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
119821547  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
119822367  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
119816292  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
119817139  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
119800371  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
119808111  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
119803343  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
119813558  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
119807886  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
119810302  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
119822816  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
119815606  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7-like [KO:K03951]
119825491  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951]
119819937  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
119807526  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
119821505  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
119810151  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial-like [KO:K03954]
119827973  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
119808788  acyl carrier protein, mitochondrial-like [KO:K03955]
119812443  acyl carrier protein, mitochondrial-like [KO:K03955]
119807454  acyl carrier protein, mitochondrial [KO:K03955]
119810775  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
119811058  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
119803407  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
119813692  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
119818574  Ndufb1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
119800598  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
119806913  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
119804078  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
119825606  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
119825940  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
119825963  Ndufb6; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
119801780  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
119811753  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
119823332  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
119812927  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
119804641  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
119804932  LOW QUALITY PROTEIN: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial-like [KO:K03934] [EC:7.1.1.2]
119820696  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
119827486  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
119814166  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
119810090  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
119809107  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
119803219  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
119810541  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
119816982  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
119818379  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
119825922  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
119810417  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
119821308  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
119814511  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
119826441  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
119802653  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
119809157  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
119816786  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
119827624  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
119809004  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
119816634  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
119822897  cytochrome c1, heme protein, mitochondrial [KO:K00413]
119810716  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
119818376  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
119817782  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
119823100  cytochrome b-c1 complex subunit 7 [KO:K00417]
119826150  cytochrome b-c1 complex subunit 7 [KO:K00417]
119812643  cytochrome b-c1 complex subunit 8 [KO:K00418]
119822943  cytochrome b-c1 complex subunit 10 [KO:K00420]
119824516  cytochrome b-c1 complex subunit 10-like [KO:K00420]
119801810  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
119815669  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
119808969  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
119820956  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
119800700  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
119823348  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
119824540  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
119813186  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
119826874  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
119821317  cytochrome c oxidase subunit 6B2 [KO:K02267]
119803616  cytochrome c oxidase subunit 6B1-like [KO:K02267]
119812348  cytochrome c oxidase subunit 6B1-like [KO:K02267]
119820632  cytochrome c oxidase subunit 6B1 [KO:K02267]
119823502  cytochrome c oxidase subunit 6C-2 [KO:K02268]
119804134  cytochrome c oxidase subunit 6C-2-like [KO:K02268]
119808129  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
119821387  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
119810497  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
119820589  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
119821824  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
119824561  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
119804752  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
119807117  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
119818750  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
119809813  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
119824069  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
119822805  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
119818361  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
119806473  cytochrome c oxidase subunit 8B, mitochondrial-like [KO:K02273]
119814908  Atp5f1a; ATP synthase subunit alpha, mitochondrial [KO:K02132]
119803617  Atp5f1b; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
119801085  ATP synthase subunit gamma, mitochondrial [KO:K02136]
119816174  Atp5f1c; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
119821561  Atp5f1d; ATP synthase subunit delta, mitochondrial [KO:K02134]
119814896  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
119800757  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
119807929  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
119827952  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
119822775  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
119818833  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
119812397  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
119826216  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
119807061  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
119817869  ATP synthase subunit d, mitochondrial-like isoform X1 [KO:K02138]
119812417  Atp5pd; ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138]
119824792  Atp5po; ATP synthase subunit O, mitochondrial [KO:K02137]
119825399  ATP synthase subunit O, mitochondrial-like [KO:K02137]
119813406  ATP synthase subunit O, mitochondrial-like [KO:K02137]
119824693  Atp5pf; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
119819422  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
119806930  proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
119816961  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
119810264  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
119826499  proteasome subunit alpha type-4-like [KO:K02728] [EC:3.4.25.1]
119814848  Psma8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
119814251  Psma7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
119800933  Psma5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
119826314  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
119807368  LOW QUALITY PROTEIN: proteasome subunit alpha type-3-like [KO:K02727] [EC:3.4.25.1]
119818710  Psma3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
119811925  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
119818701  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
119808982  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
119804953  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
119805275  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
119812338  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
119799913  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
119817964  Psmb2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
119800066  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
119821027  Psmb1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
119801014  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
119826096  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 7-like [KO:K03061]
119803931  Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061]
119819347  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
119821340  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
119800168  Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064]
119814467  Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065]
119817394  26S proteasome regulatory subunit 8-like [KO:K03066]
119811765  Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066]
119825492  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
119820512  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
119812910  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
119825311  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
119813232  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
119812065  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
119828047  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
119802238  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
119808239  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
119800726  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
119812782  26S proteasome non-ATPase regulatory subunit 4-like [KO:K03029]
119818979  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
119820678  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
119814373  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
119815375  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
119818482  complement C5-like [KO:K03994]
119810709  C6; complement component C6 [KO:K03995]
119810733  C7; complement component C7 [KO:K03996]
119817027  C8a; complement component C8 alpha chain [KO:K03997]
119817793  C8b; complement component C8 beta chain [KO:K03998]
119818902  C8g; complement component C8 gamma chain [KO:K03999]
119808702  C9; complement component C9 [KO:K04000]
119816950  C1qa; complement C1q subcomponent subunit A [KO:K03986]
119816913  C1qb; complement C1q subcomponent subunit B [KO:K03987]
119816840  C1qc; complement C1q subcomponent subunit C [KO:K03988]
119808171  heat shock cognate 71 kDa protein-like [KO:K03283]
119827066  Hspa6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
119809148  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
119822472  heat shock 70 kDa protein 1A [KO:K03283]
119822473  heat shock 70 kDa protein 1-like [KO:K03283]
119822478  heat shock 70 kDa protein 1A [KO:K03283]
119816023  heat shock cognate 71 kDa protein-like [KO:K03283]
119822758  heat shock cognate 71 kDa protein-like [KO:K03283]
119826334  heat shock cognate 71 kDa protein-like [KO:K03283]
119819233  Notch1; neurogenic locus notch homolog protein 1 [KO:K02599]
119807503  Cav1; caveolin-1 [KO:K06278]
119807487  Cav2; caveolin-2 [KO:K12958]
119808233  Cav3; caveolin-3 [KO:K12959]
119816315  Pik3cd; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
119810503  Pik3cb; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
119826317  Pik3ca; LOW QUALITY PROTEIN: phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
119808979  Pik3r1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
119817743  phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
119812102  Pik3r2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
119827346  Prkcd; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
119805427  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
119802000  cytochrome b-245 light chain [KO:K08009]
119824015  Ncf4; neutrophil cytosol factor 4 [KO:K08012]
119825197  Ncf1; LOW QUALITY PROTEIN: neutrophil cytosol factor 1 [KO:K08011]
119827668  Ncf2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
119814535  ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
119824999  Rac1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
119822752  Rac2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
119822988  Mapk11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
119823276  Mapk12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
119823839  Mapk13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
119823840  Mapk14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
119811270  Mapk9; LOW QUALITY PROTEIN: mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
119813574  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
119827427  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
119824680  Mapk1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
119813356  Mapk3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
119820446  Creb1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
119819075  Atf2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
119814789  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
119821362  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
119801303  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
119826298  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
119806715  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
119807891  Creb5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
119822471  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
119814710  Egr1; early growth response protein 1 [KO:K09203]
119811677  Ccl5; C-C motif chemokine 5 [KO:K12499]
119813991  Il1a; interleukin-1 alpha [KO:K04383]
119814887  Il1b; interleukin-1 beta [KO:K04519]
119808459  Il6; interleukin-6 [KO:K05405]
119822506  Tnf; tumor necrosis factor [KO:K03156]
119824224  Gsk3b; LOW QUALITY PROTEIN: glycogen synthase kinase-3 beta [KO:K03083] [EC:2.7.11.26]
119802199  Csnk2a2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
119814096  Csnk2a1; LOW QUALITY PROTEIN: casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
119822509  Csnk2b; casein kinase II subunit beta [KO:K03115]
119816354  Kif5b; kinesin-1 heavy chain isoform X1 [KO:K10396]
119803441  Kif5a; kinesin heavy chain isoform X1 [KO:K10396]
119819571  Kif5c; kinesin heavy chain [KO:K10396]
119822629  Klc4; kinesin light chain 4 [KO:K10407]
119819630  Klc1; kinesin light chain 1 isoform X1 [KO:K10407]
119827138  Klc2; kinesin light chain 2 [KO:K10407]
119820796  Klc3; kinesin light chain 3 isoform X1 [KO:K10407]
119821363  Tuba4a; tubulin alpha-4A chain isoform X1 [KO:K07374]
119822305  tubulin alpha-1B chain [KO:K07374]
119822381  Tuba1a; tubulin alpha-1A chain [KO:K07374]
119822383  tubulin alpha-1C chain [KO:K07374]
119817489  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
119805855  tubulin alpha-3 chain [KO:K07374]
119806400  LOW QUALITY PROTEIN: tubulin alpha-3 chain-like [KO:K07374]
119806404  LOW QUALITY PROTEIN: tubulin alpha-3 chain-like [KO:K07374]
119806674  Tuba8; tubulin alpha-8 chain [KO:K07374]
119814720  Tubb1; tubulin beta-1 chain isoform X1 [KO:K07375]
119801749  uncharacterized protein LOC119801749 [KO:K07375]
119816178  tubulin beta-2B chain [KO:K07375]
119823163  Tubb; tubulin beta chain [KO:K07375]
119819107  Tubb4b; tubulin beta-4B chain [KO:K07375]
119825449  Tubb4a; tubulin beta-4A chain [KO:K07375]
119814018  Tubb6; tubulin beta-6 chain [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.
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Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
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)
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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
  Authors
Jones E, Mead S
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Genetic risk factors for Creutzfeldt-Jakob disease.
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Neurobiol Dis 142:104973 (2020)
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Reference
PMID:18245809
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Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
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Am J Pathol 172:555-65 (2008)
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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
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
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Nature 443:803-10 (2006)
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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)
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Reference
PMID:17085779
  Authors
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
  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)
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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)
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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.
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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.
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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
  Authors
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)
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.
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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
aamp00190  Oxidative phosphorylation
aamp03050  Proteasome
aamp04020  Calcium signaling pathway
aamp04140  Autophagy - animal
aamp04141  Protein processing in endoplasmic reticulum
aamp04142  Lysosome
aamp04144  Endocytosis
aamp04210  Apoptosis
aamp04330  Notch signaling pathway
aamp04610  Complement and coagulation cascades
aamp04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system