KEGG   PATHWAY: mpah05020
Entry
mpah05020                   Pathway                                
Name
Prion disease - Mus pahari (shrew mouse)
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
mpah05020  Prion disease
mpah05020

Organism
Mus pahari (shrew mouse) [GN:mpah]
Gene
110318145  Prnp; major prion protein [KO:K05634]
110327227  Ncam1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
110329638  Ncam2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
110321144  Lamc1; laminin subunit gamma-1 [KO:K05635]
110329810  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
110316480  Stip1; stress-induced-phosphoprotein 1 [KO:K09553]
110337220  Prkaca; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
110320995  Prkacb; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
110320030  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
110326389  Fyn; tyrosine-protein kinase Fyn [KO:K05703] [EC:2.7.10.2]
110319261  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
110329701  Grin2a; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
110316284  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
110331735  Grin2c; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
110319211  Grin2d; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
110322986  Grin3a; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
110326314  Grin3b; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
110318209  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
110317086  Cacna1c; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
110325961  Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
110313596  Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
110322010  Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
110319438  Hspa5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
110324429  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
110334651  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
110326609  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
110330776  Ryr1; ryanodine receptor 1 [KO:K04961]
110334252  Ryr2; ryanodine receptor 2 [KO:K04962]
110319080  Ryr3; ryanodine receptor 3 isoform X1 [KO:K04963]
110317210  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
110317251  Itpr2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
110338229  Itpr3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
110327565  caspase-12-like [KO:K04741] [EC:3.4.22.-]
110321008  Ppp3ca; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
110325335  Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
110325367  Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
110330477  Ppp3r1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
110322987  Ppp3r2; calcineurin subunit B type 2 [KO:K06268]
110326250  Bad; bcl2-associated agonist of cell death isoform X1 [KO:K02158]
110331342  Vdac1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
110325370  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
110336305  Vdac3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
110313362  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
110336417  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
110320569  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
110325751  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
110326877  Apaf1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
110332378  cytochrome c-like [KO:K08738]
110316223  cytochrome c, somatic [KO:K08738]
110317786  cytochrome c, testis-specific [KO:K08738]
110323338  Casp9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
110336608  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
110326646  Mcu; calcium uniporter protein, mitochondrial [KO:K20858]
36165356  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
36165352  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
36165337  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
36165334  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
36165335  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
36165330  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
36165329  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
110316888  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
110335628  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
110338235  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
110313685  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
110333203  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
110337062  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
110326234  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
110317006  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
110316386  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
110335084  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
110335777  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
110318772  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
110316704  Ndufa9; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
110321748  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
110328243  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
110335867  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
110326784  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
110336643  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
110324555  Ndufb1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
110317149  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
110322308  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
110326037  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
110329959  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
110320492  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
110338914  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
110333533  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6-like [KO:K03962]
110337650  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
110331668  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
110334970  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
110338345  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
110314570  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
110321204  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
110321406  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
110318572  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
110329019  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
110323693  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
110329167  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
110326881  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
110327359  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
110320722  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
110332408  NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
110328746  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
110323139  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
110322178  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
110324197  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like isoform X1 [KO:K00237]
110328112  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
110333870  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
36165327  CYTB; cytochrome b [KO:K00412]
110334724  cytochrome c1, heme protein, mitochondrial [KO:K00413]
110327331  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
110318232  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
110323660  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
110334014  cytochrome b-c1 complex subunit 7 [KO:K00417]
110332489  cytochrome b-c1 complex subunit 8 [KO:K00418]
110330872  cytochrome b-c1 complex subunit 9 [KO:K00419]
110326979  cytochrome b-c1 complex subunit 10 [KO:K00420]
36165339  COX3; cytochrome c oxidase subunit III [KO:K02262]
36165346  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
36165343  COX2; cytochrome c oxidase subunit II [KO:K02261]
110319299  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
110337554  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
110328046  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
110322258  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
110313018  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
110325548  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
110332943  cytochrome c oxidase subunit 6B1 [KO:K02267]
110336798  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
110335202  cytochrome c oxidase subunit 6C [KO:K02268]
110328169  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
110338814  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
110335711  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
110313979  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
110330226  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
110329085  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
110332346  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
110324960  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
110339130  cytochrome c oxidase subunit 8B, mitochondrial [KO:K02273]
110333376  Atp5a1; ATP synthase subunit alpha, mitochondrial isoform X1 [KO:K02132]
110324657  ATP synthase subunit beta, mitochondrial-like [KO:K02133] [EC:7.1.2.2]
110326266  Atp5b; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
110333647  Atp5c1; ATP synthase subunit gamma, mitochondrial [KO:K02136]
110326670  Atp5d; ATP synthase subunit delta, mitochondrial isoform X1 [KO:K02134]
110319359  Atp5e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
36165340  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
110320209  Atp5f1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
110318554  Atp5g3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128]
110331847  Atp5g1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
110334965  Atp5g2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
110320480  ATP synthase subunit d, mitochondrial-like isoform X1 [KO:K02138]
110332361  Atp5h; ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138]
110329905  Atp5o; ATP synthase subunit O, mitochondrial [KO:K02137]
110329594  Atp5j; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
36165341  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
110324196  Psma6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
110333899  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
110327417  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
110319566  Psma7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
110333269  Psma8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
110320113  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
110321258  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
110324319  Psma3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
110331969  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
110317854  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
110332263  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
110323478  Psmb2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
110325463  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
110338158  Psmb1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
110320096  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
110317419  Psmc2; 26S protease regulatory subunit 7 [KO:K03061]
110324155  Psmc1; 26S protease regulatory subunit 4 isoform X1 [KO:K03062]
110317526  Psmc4; 26S protease regulatory subunit 6B isoform X1 [KO:K03063]
110325226  Psmc6; 26S protease regulatory subunit 10B [KO:K03064]
110317665  Psmc3; 26S protease regulatory subunit 6A [KO:K03065]
110331458  Psmc5; 26S protease regulatory subunit 8 [KO:K03066]
110329924  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
110321309  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
110332775  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
110312964  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
110331343  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
110331208  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
110325295  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
110337398  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
110319108  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
110318110  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
110323088  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
110318228  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
110319004  proteasomal ubiquitin receptor ADRM1-like [KO:K06691]
110317098  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
110317539  C5; complement C5 [KO:K03994]
110329165  C6; complement component C6 [KO:K03995]
110328891  C7; complement component C7 [KO:K03996]
110323615  C8a; complement component C8 alpha chain [KO:K03997]
110323447  C8b; complement component C8 beta chain isoform X1 [KO:K03998]
110318150  C8g; complement component C8 gamma chain [KO:K03999]
110329162  C9; complement component C9 [KO:K04000]
110323698  C1qa; complement C1q subcomponent subunit A [KO:K03986]
110323604  C1qb; complement C1q subcomponent subunit B [KO:K03987]
110323611  C1qc; complement C1q subcomponent subunit C [KO:K03988]
110335695  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
110335998  heat shock 70 kDa protein 1A [KO:K03283]
110327293  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
110318758  Notch1; neurogenic locus notch homolog protein 1 [KO:K02599]
110315910  Cav1; caveolin-1 [KO:K06278]
110315911  Cav2; caveolin-2 [KO:K12958]
110317056  Cav3; caveolin-3 [KO:K12959]
110323448  Pik3cd; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
110319750  Pik3ca; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
110327251  Pik3cb; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
110328811  Pik3r1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
110336420  Pik3r2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
110322865  Pik3r3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
110326026  Prkcd; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
110313555  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
110337603  cytochrome b-245 light chain isoform X1 [KO:K08009]
110335015  Ncf4; neutrophil cytosol factor 4 [KO:K08012]
110312979  Ncf1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
110321813  Ncf2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
110339030  Rac1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
110334678  Rac2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
110334495  Mapk11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
110337896  Mapk13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
110337946  Mapk14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
110334829  Mapk12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
110330449  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
110332652  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
110325466  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
110329343  Mapk1; mitogen-activated protein kinase 1 isoform X1 [KO:K04371] [EC:2.7.11.24]
110320020  Mapk3; mitogen-activated protein kinase 3 isoform X1 [KO:K04371] [EC:2.7.11.24]
110322391  Creb1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
110317821  Atf2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
110320678  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
110316152  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
110326077  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
110338975  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
110318364  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
110315994  Creb5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
110335526  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
110333149  Egr1; early growth response protein 1 [KO:K09203]
110332476  Ccl5; C-C motif chemokine 5 [KO:K12499]
110319393  Il1a; interleukin-1 alpha [KO:K04383]
110319321  Il1b; interleukin-1 beta isoform X1 [KO:K04519]
110317140  Il6; interleukin-6 [KO:K05405]
110335791  Tnf; tumor necrosis factor isoform X1 [KO:K03156]
110329614  Gsk3b; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
110317612  Csnk2a1; casein kinase II subunit alpha isoform X1 [KO:K03097] [EC:2.7.11.1]
110337286  Csnk2a2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
110335376  Csnk2b; casein kinase II subunit beta isoform X1 [KO:K03115]
110332910  Kif5b; kinesin-1 heavy chain [KO:K10396]
110326230  Kif5a; kinesin heavy chain [KO:K10396]
110318104  Kif5c; kinesin heavy chain [KO:K10396]
110319541  Klc2; kinesin light chain 2 [KO:K10407]
110336348  Klc3; kinesin light chain 3 isoform X1 [KO:K10407]
110324984  Klc1; kinesin light chain 1 isoform X1 [KO:K10407]
110335434  Klc4; kinesin light chain 4 isoform X1 [KO:K10407]
110316306  tubulin alpha-3 chain isoform X1 [KO:K07374]
110321284  Tuba4a; tubulin alpha-4A chain isoform X1 [KO:K07374]
110334563  tubulin alpha-1C chain-like [KO:K07374]
110316395  Tuba8; tubulin alpha-8 chain isoform X1 [KO:K07374]
110316417  tubulin alpha-3 chain isoform X1 [KO:K07374]
110334627  tubulin alpha-1A chain-like [KO:K07374]
110325235  tubulin alpha chain-like [KO:K07374]
110334021  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
110319104  Tubb1; tubulin beta-1 chain [KO:K07375]
110333011  Tubb6; tubulin beta-6 chain isoform X1 [KO:K07375]
110335873  Tubb; tubulin beta chain isoform X1 [KO:K07375]
110335384  Tubb4a; tubulin beta-4A chain isoform X1 [KO:K07375]
110337799  Tubb3; tubulin beta-3 chain isoform X1 [KO:K07375]
110333774  tubulin beta-2A chain-like [KO:K07375]
110319452  Tubb4b; tubulin beta-4B chain isoform X1 [KO:K07375]
110333829  LOW QUALITY PROTEIN: tubulin beta-2B chain-like [KO:K07375]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
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Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
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Expert Rev Mol Med 18:e5 (2016)
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PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
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Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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Soto C, Satani N
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The intricate mechanisms of neurodegeneration in prion diseases.
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Trends Mol Med 17:14-24 (2011)
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Reference
PMID:27060771
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The stress of prion disease.
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Brain Res 1648:553-560 (2016)
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PMID:32565065
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Genetic risk factors for Creutzfeldt-Jakob disease.
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Neurobiol Dis 142:104973 (2020)
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PMID:18245809
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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
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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)
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Reference
PMID:17051207
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Prions and their partners in crime.
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Nature 443:803-10 (2006)
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PMID:11592845
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Chiesa R, Harris DA
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Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
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Reference
PMID:17085779
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Fasano C, Campana V, Zurzolo C
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Prions: protein only or something more? Overview of potential prion cofactors.
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J Mol Neurosci 29:195-214 (2006)
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PMID:14705136
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Neuroprotective functions of prion protein.
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J Neurosci Res 75:153-61 (2004)
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PMID:10398674
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Harris DA
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Cellular biology of prion diseases.
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Clin Microbiol Rev 12:429-44 (1999)
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PMID:15890824
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Prion biology relevant to bovine spongiform encephalopathy.
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J Anim Sci 83:1455-76 (2005)
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PMID:27581199
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Peggion C, Bertoli A, Sorgato MC
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Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
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Biochem Biophys Res Commun 483:1148-1155 (2017)
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PMID:31618844
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Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
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Prion Protein in Glioblastoma Multiforme.
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Int J Mol Sci 20:E5107 (2019)
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PMID:31795242
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Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
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The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
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Int J Mol Sci 20:E6004 (2019)
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Reference
PMID:30710214
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Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
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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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PMID:23439666
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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)
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PMID:28643372
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Halliday M, Hughes D, Mallucci GR
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Fine-tuning PERK signaling for neuroprotection.
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J Neurochem 142:812-826 (2017)
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PMID:29194372
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Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
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Pathogens 6:E63 (2017)
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Reference
PMID:24619348
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Hetz C, Mollereau B
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Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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Reference
PMID:29476642
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Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
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FEBS J 286:342-355 (2019)
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Reference
PMID:21722302
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Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
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Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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J Cell Mol Med 15:2025-39 (2011)
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PMID:18420416
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Ferreiro E, Oliveira CR, Pereira CM
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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)
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Reference
PMID:16844381
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An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
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Neurobiol Dis 23:669-78 (2006)
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PMID:21980554
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Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
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Commun Integr Biol 4:258-61 (2011)
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Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
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Exp Mol Med 47:e147 (2015)
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Disease-associated prion protein oligomers inhibit the 26S proteasome.
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Mol Cell 26:175-88 (2007)
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PMID:10195903
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Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Science 284:339-43 (1999)
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PMID:14532116
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Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
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EMBO J 22:5435-45 (2003)
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PMID:31417367
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Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
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Front Cell Neurosci 13:350 (2019)
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PMID:29261664
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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
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Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
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PLoS One 12:e0188340 (2017)
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Reference
PMID:21196165
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Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
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Understanding the neurospecificity of Prion protein signaling.
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Front Biosci (Landmark Ed) 16:169-86 (2011)
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Reference
PMID:23479001
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Prion protein and its role in signal transduction.
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Cell Mol Biol Lett 18:209-30 (2013)
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PMID:28507517
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The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
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Front Aging Neurosci 9:120 (2017)
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PMID:15528202
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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.
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J Biol Chem 280:1529-34 (2005)
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PMID:15640354
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  Title
Notch-1 activation and dendritic atrophy in prion disease.
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Proc Natl Acad Sci U S A 102:886-91 (2005)
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Reference
PMID:20205843
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Dearmond SJ, Bajsarowicz K
  Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
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Mol Neurodegener 5:6 (2010)
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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.
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Mol Neurobiol 56:2159-2173 (2019)
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Reference
PMID:28714865
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Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
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J Clin Invest 127:3230-3239 (2017)
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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
mpah00190  Oxidative phosphorylation
mpah03050  Proteasome
mpah04020  Calcium signaling pathway
mpah04140  Autophagy - animal
mpah04141  Protein processing in endoplasmic reticulum
mpah04142  Lysosome
mpah04144  Endocytosis
mpah04210  Apoptosis
mpah04330  Notch signaling pathway
mpah04610  Complement and coagulation cascades
mpah04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system