KEGG   PATHWAY: eai05020
Entry
eai05020                    Pathway                                
Name
Prion disease - Equus asinus (ass)
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
eai05020  Prion disease
eai05020

Organism
Equus asinus (ass) [GN:eai]
Gene
106847681  major prion protein [KO:K05634]
106840225  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
106848210  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
106837014  LAMC1; laminin subunit gamma-1 [KO:K05635]
106847316  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
106833637  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
106844316  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
106824036  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
106838268  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
106847048  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
106822778  GRIN1; glutamate receptor ionotropic, NMDA 1 [KO:K05208]
106836764  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
106826757  GRIN2B; glutamate receptor ionotropic, NMDA 2B isoform X1 [KO:K05210]
106830197  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
106838305  GRIN2D; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D [KO:K05212]
106831779  GRIN3A; glutamate receptor ionotropic, NMDA 3A [KO:K05213]
106834234  GRIN3B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 3B [KO:K05214]
106822757  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
106839455  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
106822066  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X8 [KO:K04851]
106826963  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
106823553  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X3 [KO:K04857]
106830826  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
106838571  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
106827800  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
106842437  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
106833003  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
106836101  RYR1; LOW QUALITY PROTEIN: ryanodine receptor 1 [KO:K04961]
106834687  RYR2; ryanodine receptor 2 isoform X4 [KO:K04962]
106835144  RYR3; LOW QUALITY PROTEIN: ryanodine receptor 3 [KO:K04963]
106848450  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
106834887  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
106834634  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
106837654  caspase-12-like [KO:K04741] [EC:3.4.22.-]
106847712  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X4 [KO:K04348] [EC:3.1.3.16]
106834431  PPP3CA; LOW QUALITY PROTEIN: serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
106842388  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X3 [KO:K04348] [EC:3.1.3.16]
106828340  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
106831788  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
106833626  BAD; bcl2-associated agonist of cell death [KO:K02158]
106834658  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
106837945  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
106843557  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
106828962  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
106841502  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
106842512  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
106830087  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
106829894  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
106828596  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
106827252  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
106829744  cytochrome c [KO:K08738]
106826627  cytochrome c, testis-specific [KO:K08738]
106828005  CASP9; caspase-9 isoform X2 [KO:K04399] [EC:3.4.22.62]
106830081  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
106842371  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
808061  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
808059  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
808060  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
808055  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
808056  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
808058  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
808057  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
106833485  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
106828659  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
106829925  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
106845129  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
106823773  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
106840869  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
106848374  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
106832994  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
106834450  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
106829331  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
106829867  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
106848494  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
106844493  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X5 [KO:K03951]
106837867  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
106831495  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
106832206  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
106846147  NDUFAB1; acyl carrier protein, mitochondrial isoform X2 [KO:K03955]
106832748  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
106831170  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
106825466  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
106825375  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
106842621  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
106823500  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
106823734  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
106829307  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
106841469  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
106844306  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 isoform X2 [KO:K03963]
106846749  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
106839361  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
106836226  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
106847989  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
106828149  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
106827589  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
106844019  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
106843348  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
106830578  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 isoform X2 [KO:K03938]
106835886  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
106834185  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
106844280  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
106838418  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
106842890  NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
106835869  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
106827983  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
106827584  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
106840217  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
106834965  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
808065  CYTB; cytochrome b [KO:K00412]
106841912  cytochrome c1, heme protein, mitochondrial [KO:K00413]
106830662  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
106841746  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
106822600  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
106835419  cytochrome b-c1 complex subunit 7 [KO:K00417]
106841008  cytochrome b-c1 complex subunit 8 [KO:K00418]
106827325  cytochrome b-c1 complex subunit 9 [KO:K00419]
106834176  cytochrome b-c1 complex subunit 10 [KO:K00420]
808054  COX3; cytochrome c oxidase subunit III [KO:K02262]
808066  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
808064  COX2; cytochrome c oxidase subunit II [KO:K02261]
106829176  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
106833711  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
106831275  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
106842542  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
106831875  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
106844790  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
106829247  cytochrome c oxidase subunit 6B1 [KO:K02267]
106842364  cytochrome c oxidase subunit 6C [KO:K02268]
123285498  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6C-like [KO:K02268]
106842554  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
106848636  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
106841238  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
106846922  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
106841203  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
106833641  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
123286886  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
106823353  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
106832969  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
106831232  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
106834227  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
106824394  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
808063  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
106828604  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
106828058  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
106837906  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128]
106826529  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
106830277  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
106832829  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
106842470  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
808062  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
106838802  PSMA6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
106831254  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
106840832  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
106836405  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
106822876  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
106827395  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
106833076  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
106845859  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
106844150  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
106830813  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
106826516  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
123276027  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
106830527  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
106837200  proteasome subunit beta type-2-like [KO:K02734] [EC:3.4.25.1]
106840650  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
106827778  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
106846505  proteasome subunit beta type-4-like [KO:K02736] [EC:3.4.25.1]
106848364  proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
106825237  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
106825368  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
106843286  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
106826770  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
106844015  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
106835533  PSMC5; 26S proteasome regulatory subunit 8 isoform X2 [KO:K03066]
106834518  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
106827890  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
106826388  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
106842301  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
106835560  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
106844623  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
106842647  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
106842138  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
106843856  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
106846512  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
106843144  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X2 [KO:K03030]
106836062  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
106838407  LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
106842713  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
106824223  C5; complement C5 isoform X1 [KO:K03994]
106847872  C6; complement component C6 [KO:K03995]
106847873  C7; LOW QUALITY PROTEIN: complement component C7 [KO:K03996]
106833473  C8A; complement component C8 alpha chain isoform X2 [KO:K03997]
106833472  C8B; complement component C8 beta chain [KO:K03998]
106845531  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
106824775  C9; complement component C9 [KO:K04000]
106839999  C1QA; complement C1q subcomponent subunit A [KO:K03986]
106839997  C1QB; complement C1q subcomponent subunit B [KO:K03987]
106839998  C1QC; complement C1q subcomponent subunit C [KO:K03988]
106832775  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
106822285  heat shock 70 kDa protein 1 [KO:K03283]
106831112  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
106825061  heat shock 70 kDa protein 1-like [KO:K03283]
123282797  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
123288213  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1-like [KO:K03283]
106845559  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
106829272  CAV1; caveolin-1 isoform X1 [KO:K06278]
106829266  CAV2; caveolin-2 [KO:K12958]
106841765  CAV3; caveolin-3 [KO:K12959]
106838100  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
106829313  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
106836695  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
106840141  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X2 [KO:K02649]
106822593  phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
106824993  PIK3R2; LOW QUALITY PROTEIN: phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
106822994  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
106825146  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
106828257  cytochrome b-245 light chain [KO:K08009]
106837573  NCF4; neutrophil cytosol factor 4 [KO:K08012]
106840982  NCF1; neutrophil cytosol factor 1 [KO:K08011]
106836994  NCF2; neutrophil cytosol factor 2 [KO:K08010]
106840518  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
106837585  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
106824547  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
106824582  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
106846005  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
106846006  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
106839421  MAPK10; mitogen-activated protein kinase 10 isoform X2 [KO:K04440] [EC:2.7.11.24]
106831384  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
106843729  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
106845632  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
106848262  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
106828159  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
106837905  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X2 [KO:K04450]
106846249  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X2 [KO:K09048]
106843990  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
106846981  CREB3; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3 [KO:K09048]
106829982  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
106832323  CREB3L3; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
106831046  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
106831009  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
106839216  EGR1; early growth response protein 1 [KO:K09203]
106844660  CCL5; C-C motif chemokine 5 [KO:K12499]
106838556  IL1A; interleukin-1 alpha [KO:K04383]
106838557  LOW QUALITY PROTEIN: interleukin-1 beta [KO:K04519]
123286234  interleukin-1 beta-like [KO:K04519]
123286693  interleukin-1 beta-like [KO:K04519]
106824372  IL6; interleukin-6 [KO:K05405]
106825034  TNF; tumor necrosis factor isoform X1 [KO:K03156]
106829188  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
106822212  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
106833699  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
106825044  CSNK2B; casein kinase II subunit beta isoform X1 [KO:K03115]
106833007  KIF5A; kinesin heavy chain [KO:K10396]
106837958  KIF5B; kinesin-1 heavy chain [KO:K10396]
106825084  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
106839611  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
106843755  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
106828722  KLC4; kinesin light chain 4 [KO:K10407]
106833539  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
106834033  LOW QUALITY PROTEIN: tubulin alpha-3 chain [KO:K07374]
106836806  LOW QUALITY PROTEIN: tubulin alpha-1A chain [KO:K07374]
106836811  tubulin alpha-1B chain [KO:K07374]
106839445  TUBA8; tubulin alpha-8 chain isoform X2 [KO:K07374]
106840555  LOW QUALITY PROTEIN: tubulin alpha-1D chain-like [KO:K07374]
106840558  tubulin alpha-4A chain [KO:K07374]
106844743  tubulin alpha-3 chain [KO:K07374]
106826191  LOW QUALITY PROTEIN: P2X purinoceptor 6 [KO:K07374]
106833825  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
123275613  tubulin alpha-1C chain isoform X1 [KO:K07374]
106823414  TUBB6; tubulin beta-6 chain [KO:K07375]
106832761  TUBB4A; tubulin beta-4A chain [KO:K07375]
106825507  TUBB; tubulin beta chain [KO:K07375]
106824393  TUBB1; tubulin beta-1 chain [KO:K07375]
106828225  TUBB3; tubulin beta-3 chain [KO:K07375]
106847879  tubulin beta-2A chain [KO:K07375]
106847891  LOW QUALITY PROTEIN: tubulin beta-2B chain [KO:K07375]
106822767  TUBB4B; tubulin beta-4B 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.
  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.
  Journal
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.
  Journal
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.
  Journal
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
eai00190  Oxidative phosphorylation
eai03050  Proteasome
eai04020  Calcium signaling pathway
eai04140  Autophagy - animal
eai04141  Protein processing in endoplasmic reticulum
eai04142  Lysosome
eai04144  Endocytosis
eai04210  Apoptosis
eai04330  Notch signaling pathway
eai04610  Complement and coagulation cascades
eai04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system