KEGG   PATHWAY: oaa05020
Entry
oaa05020                    Pathway                                
Name
Prion disease - Ornithorhynchus anatinus (platypus)
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
oaa05020  Prion disease
oaa05020

Organism
Ornithorhynchus anatinus (platypus) [GN:oaa]
Gene
100086593  major prion protein isoform X1 [KO:K05634]
100081069  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
100085373  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
100085738  LAMC1; LOW QUALITY PROTEIN: laminin subunit gamma-1 [KO:K05635]
114817987  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100080264  STIP1; stress-induced-phosphoprotein 1 isoform X1 [KO:K09553]
100078846  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
100088079  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
114814649  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
100073798  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
100082705  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
100074436  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
100090612  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100681823  GRIN2C; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2C [KO:K05211]
100084653  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
100080844  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
100092855  GRIN3B; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
100681261  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
100082618  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
100077296  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851]
100075939  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
100093447  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
100681083  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
100081193  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
100074113  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100089902  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
103167225  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
100681936  RYR1; ryanodine receptor 1 [KO:K04961]
100084273  RYR2; LOW QUALITY PROTEIN: ryanodine receptor 2 [KO:K04962]
100089020  RYR3; LOW QUALITY PROTEIN: ryanodine receptor 3 [KO:K04963]
100075749  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
103164954  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
100088770  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
100087467  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
100083890  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
100073776  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
100083665  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
114810030  BAD; bcl2-associated agonist of cell death [KO:K02158]
100079428  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100075011  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
100076584  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
100080434  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100087351  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
114812883  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100073904  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
100073402  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
100077443  cytochrome c [KO:K08738]
100092345  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
100087105  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100077394  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
808708  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
808700  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
808710  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
808699  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
808698  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
808701  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
808706  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100092852  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100076572  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100080859  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X2 [KO:K03944]
100078945  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100088525  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
103169972  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
100089801  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
114814719  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100076349  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100086818  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
103171189  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100081651  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
100085363  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
100080496  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
100073665  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100082311  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
100073802  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100682250  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
103167180  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100086429  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100089265  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100089450  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100083885  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
100680990  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
114813135  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100084515  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
100080588  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100081152  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
114812795  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
100085312  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
114807989  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
100082649  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100075551  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
100076967  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100081627  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100076603  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
114809999  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100092030  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
114805804  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
100083131  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
100074487  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
100089418  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
114815211  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
100074926  cytochrome b-c1 complex subunit Rieske, mitochondrial isoform X1 [KO:K00411] [EC:7.1.1.8]
808703  CYTB; cytochrome b [KO:K00412]
107546954  cytochrome c1, heme protein, mitochondrial [KO:K00413]
100074417  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
100077249  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
114818207  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
100073821  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
100080559  cytochrome b-c1 complex subunit 8 [KO:K00418]
100088975  cytochrome b-c1 complex subunit 9 [KO:K00419]
100082774  UQCR11; cytochrome b-c1 complex subunit 10 [KO:K00420]
808707  COX3; cytochrome c oxidase subunit III [KO:K02262]
808704  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
808709  COX2; cytochrome c oxidase subunit II [KO:K02261]
100076685  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
114806037  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
100081055  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
100091270  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
100079440  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
114809040  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
114809154  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100089037  cytochrome c oxidase subunit 6B1 [KO:K02267]
100074669  cytochrome c oxidase subunit 6C [KO:K02268]
100076020  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100091985  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
114811991  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
100073487  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100077093  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
100085791  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
100075472  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100087676  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100680772  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100090214  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100074047  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
808705  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100076804  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100084526  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100090032  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
114814979  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100085769  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
100083165  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
114817674  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
808702  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100077605  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
100075308  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100082413  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
100073396  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100086434  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100093532  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100086673  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
114808032  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100078811  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100087357  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100077529  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100089973  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100082504  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100091687  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100076569  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100088021  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100088836  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100093302  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100080509  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
114815139  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
100076043  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100077344  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100080824  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100074601  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
100079412  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
103166362  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100092874  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100078921  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100092761  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100077210  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
100073491  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100074418  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
100093442  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
100681650  C5; complement C5 [KO:K03994]
100075246  C6; complement component C6 isoform X1 [KO:K03995]
100088665  C7; complement component C7 isoform X1 [KO:K03996]
100090039  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
100081005  C8B; complement component C8 beta chain [KO:K03998]
100075936  C9; complement component C9 [KO:K04000]
114812254  C1QB; complement C1q subcomponent subunit B [KO:K03987]
100089335  C1QA; complement C1q subcomponent subunit A [KO:K03988]
100080523  complement C1q subcomponent subunit C [KO:K03988]
100080044  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
100091897  heat shock 70 kDa protein 1B [KO:K03283]
114808021  LOW QUALITY PROTEIN: heat shock 70 kDa protein 1A-like [KO:K03283]
100092718  NOTCH1; neurogenic locus notch homolog protein 1 isoform X2 [KO:K02599]
100079536  CAV1; caveolin-1 [KO:K06278]
100091379  caveolin-2 [KO:K12958]
114814756  CAV2; caveolin-2 [KO:K12958]
114806542  CAV3; caveolin-3 [KO:K12959]
100086270  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
100073788  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
100074273  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
100080187  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta isoform X1 [KO:K02649]
100082075  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
103167126  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha [KO:K02649]
100088214  PRKCD; protein kinase C delta type isoform X3 [KO:K06068] [EC:2.7.11.13]
100082988  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
100084242  cytochrome b-245 light chain [KO:K08009]
100086183  NCF4; neutrophil cytosol factor 4 isoform X1 [KO:K08012]
100088825  NCF1; neutrophil cytosol factor 1 [KO:K08011]
100085817  NCF2; neutrophil cytosol factor 2 [KO:K08010]
100076220  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
100092844  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
100078507  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
100091330  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
100092264  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
100093026  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
100073424  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100076033  MAPK8; mitogen-activated protein kinase 8 isoform X2 [KO:K04440] [EC:2.7.11.24]
100082590  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
100085480  MAPK1; mitogen-activated protein kinase 1 isoform X1 [KO:K04371] [EC:2.7.11.24]
100086487  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
100093310  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
100077942  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
100078176  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100092528  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
100076562  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
103165821  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100080655  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X2 [KO:K09047]
100080771  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
100087606  EGR1; early growth response protein 1 [KO:K09203]
103165286  interleukin-1 beta [KO:K04519]
100083298  IL6; interleukin-6 [KO:K05405]
100085643  TNF; tumor necrosis factor [KO:K03156]
100092089  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
100074583  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
100079695  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
114808033  CSNK2B; casein kinase II subunit beta isoform X1 [KO:K03115]
100076805  KIF5B; kinesin-1 heavy chain [KO:K10396] [EC:5.6.1.3]
100088788  KIF5C; kinesin heavy chain [KO:K10396] [EC:5.6.1.3]
103166538  KIF5A; kinesin heavy chain isoform X1 [KO:K10396] [EC:5.6.1.3]
100075996  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
100077087  KLC4; kinesin light chain 4 [KO:K10407]
114810131  KLC2; kinesin light chain 2 [KO:K10407]
114812115  KLC3; kinesin light chain 3 [KO:K10407]
100079742  TUBA1B; tubulin alpha-1B chain [KO:K07374]
100075816  tubulin alpha-8 chain [KO:K07374]
100079918  TUBA1A; tubulin alpha-1A chain [KO:K07374]
114811984  TUBA4A; tubulin alpha-4A chain [KO:K07374]
100088515  tubulin alpha-3 chain [KO:K07374]
100085942  tubulin alpha-4A chain [KO:K07374]
100077627  tubulin alpha-8 chain isoform X2 [KO:K07374]
100079040  tubulin beta-2A chain isoform X1 [KO:K07375]
100080473  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
100086969  TUBB4A; tubulin beta-4A chain [KO:K07375]
100089091  TUBB6; tubulin beta-6 chain [KO:K07375]
100080031  TUBB3; tubulin beta-3 chain [KO:K07375]
100077354  TUBB4B; tubulin beta-4B chain [KO:K07375]
100079562  tubulin beta-2B chain [KO:K07375]
107546818  TUBB; tubulin beta chain [KO:K07375]
114805411  tubulin beta-2B chain [KO:K07375]
114805389  tubulin beta-2A chain isoform X2 [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.
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Brain Res 1648:553-560 (2016)
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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.
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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.
  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?
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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.
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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.
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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.
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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.
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Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
PMID:28643372
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
PMID:29194372
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
  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.
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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
oaa00190  Oxidative phosphorylation
oaa03050  Proteasome
oaa04020  Calcium signaling pathway
oaa04140  Autophagy - animal
oaa04141  Protein processing in endoplasmic reticulum
oaa04142  Lysosome
oaa04144  Endocytosis
oaa04210  Apoptosis
oaa04330  Notch signaling pathway
oaa04610  Complement and coagulation cascades
oaa04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

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