KEGG   PATHWAY: rno05020
rno05020                    Pathway                                

Prion disease - Rattus norvegicus (rat)
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.
Human Diseases; Neurodegenerative disease
Pathway map
rno05020  Prion disease

Rattus norvegicus (rat) [GN:rno]
24686  Prnp; major prion protein precursor [KO:K05634]
24586  Ncam1; neural cell adhesion molecule 1 precursor [KO:K06491]
288280  Ncam2; neural cell adhesion molecule 2 precursor [KO:K06491]
117036  Lamc1; laminin subunit gamma-1 precursor [KO:K05635]
24786  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:]
192277  Stip1; stress-induced-phosphoprotein 1 [KO:K09553]
25636  Prkaca; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:]
293508  Prkacb; cAMP-dependent protein kinase catalytic subunit beta [KO:K04345] [EC:]
24887  Bax; apoptosis regulator BAX [KO:K02159]
25150  Fyn; tyrosine-protein kinase Fyn [KO:K05703] [EC:]
24408  Grin1; glutamate receptor ionotropic, NMDA 1 isoform 1a precursor [KO:K05208]
24409  Grin2a; glutamate receptor ionotropic, NMDA 2A precursor [KO:K05209]
24410  Grin2b; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210]
24411  Grin2c; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
24412  Grin2d; glutamate receptor ionotropic, NMDA 2D precursor [KO:K05212]
191573  Grin3a; glutamate receptor ionotropic, NMDA 3A isoform 1 precursor [KO:K05213]
170796  Grin3b; glutamate receptor ionotropic, NMDA 3B precursor [KO:K05214]
257648  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform 1 [KO:K04849]
24239  Cacna1c; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
29716  Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851]
114493  Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
682930  Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
25617  Hspa5; endoplasmic reticulum chaperone BiP precursor [KO:K09490] [EC:]
29702  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 precursor [KO:K08860] [EC:]
54318  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
79255  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
29467  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
114207  Ryr1; ryanodine receptor 1 isoform X1 [KO:K04961]
689560  Ryr2; ryanodine receptor 2 isoform 1 [KO:K04962]
170546  Ryr3; LOW QUALITY PROTEIN: ryanodine receptor 3 [KO:K04963]
25262  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform 3 [KO:K04958]
81678  Itpr2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
25679  Itpr3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
156117  Casp12; caspase-12 [KO:K04741] [EC:3.4.22.-]
24674  Ppp3ca; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:]
24675  Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform [KO:K04348] [EC:]
171378  Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform [KO:K04348] [EC:]
29748  Ppp3r1; calcineurin subunit B type 1 [KO:K06268]
29749  Ppp3r2; calcineurin subunit B type 2 [KO:K06268]
64639  Bad; bcl2-associated agonist of cell death [KO:K02158]
83529  Vdac1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
83531  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
83532  Vdac3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
25176  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
85333  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
689108  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
282819  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial precursor [KO:K09565] [EC:]
78963  Apaf1; apoptotic protease-activating factor 1 [KO:K02084]
25309  Cycs; cytochrome c, somatic [KO:K08738]
25310  Cyct; cytochrome c, testis-specific [KO:K08738]
100363502  cytochrome c, somatic [KO:K08738]
58918  Casp9; caspase-9 [KO:K04399] [EC:]
25402  Casp3; caspase-3 [KO:K02187] [EC:]
294560  Mcu; calcium uniporter protein, mitochondrial precursor [KO:K20858]
26193  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:]
26194  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:]
26199  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:]
26201  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:]
26200  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:]
26202  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:]
26203  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:]
293655  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:]
81728  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial precursor [KO:K03943] [EC:]
64539  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform 2 [KO:K03944]
363441  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
291660  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
691001  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
684509  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
681024  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100362331  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
25488  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
315167  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
299643  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
296658  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
362440  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
316632  Ndufa10l1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10-like [KO:K03954]
678759  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial precursor [KO:K03954]
293453  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
301123  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
299739  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100911483  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
362344  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
301427  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
288088  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
294964  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
297990  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
361385  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
293991  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
299954  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
681418  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
299310  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
301458  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial precursor [KO:K03934] [EC:]
289218  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial precursor [KO:K03935] [EC:]
295923  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:]
499529  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial precursor [KO:K03937]
362588  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100363268  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
29478  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial precursor [KO:K03939]
679739  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
362837  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:]
293652  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:]
689938  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
293130  Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
157074  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial precursor [KO:K00234] [EC:]
298596  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235] [EC:]
289217  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
363061  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
291103  Uqcrfs1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:]
26192  CYTB; cytochrome b [KO:K00412]
300047  Cyc1; cytochrome c-1 [KO:K00413]
301011  Uqcrc1; cytochrome b-c1 complex subunit 1, mitochondrial precursor [KO:K00414]
293448  Uqcrc2; cytochrome b-c1 complex subunit 2, mitochondrial precursor [KO:K00415]
366448  Uqcrh; cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
362897  Uqcrb; cytochrome b-c1 complex subunit 7 [KO:K00417]
497902  Uqcrq; cytochrome b-c1 complex subunit 8 [KO:K00418]
685322  Uqcr10; ubiquinol-cytochrome c reductase complex 7.2kDa protein [KO:K00419]
100361126  RGD2320734; cytochrome b-c1 complex subunit 9 [KO:K00419]
690848  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
26204  COX3; cytochrome c oxidase subunit III [KO:K02262]
26195  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:]
26198  COX2; cytochrome c oxidase subunit II [KO:K02261]
84683  Cox4i2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform 2 precursor [KO:K02263]
29445  Cox4i1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial precursor [KO:K02263]
252934  Cox5a; cytochrome c oxidase subunit 5A, mitochondrial precursor [KO:K02264]
94194  Cox5b; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265]
25278  Cox6a2; cytochrome c oxidase subunit 6A2, mitochondrial precursor [KO:K02266]
25282  Cox6a1; cytochrome c oxidase subunit 6A1, mitochondrial precursor [KO:K02266]
688869  Cox6b1; cytochrome c oxidase subunit 6B1 [KO:K02267]
654441  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
54322  Cox6c; cytochrome c oxidase subunit 6C-2 [KO:K02268]
29507  Cox7a2; cytochrome c oxidase subunit 7A2, mitochondrial precursor [KO:K02270]
298762  Cox7a2l; cytochrome c oxidase subunit 7A2 like [KO:K02270]
688386  Cox7a2l2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
687508  cytochrome c oxidase subunit 7A1, mitochondrial isoform X2 [KO:K02270]
303393  Cox7b; cytochrome c oxidase subunit 7B, mitochondrial precursor [KO:K02271]
100361960  Cox7b2; cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100188937  Cox7c; cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
25250  Cox8b; cytochrome c oxidase subunit 8B, mitochondrial precursor [KO:K02273]
360229  Cox8c; cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
171335  Cox8a; cytochrome c oxidase subunit 8A, mitochondrial precursor [KO:K02273]
65262  Atp5f1a; ATP synthase subunit alpha, mitochondrial precursor [KO:K02132]
171374  Atp5f1b; ATP synthase subunit beta, mitochondrial precursor [KO:K02133] [EC:]
116550  Atp5f1c; ATP synthase subunit gamma, mitochondrial [KO:K02136]
245965  Atp5f1d; ATP synthase subunit delta, mitochondrial precursor [KO:K02134]
245958  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
26197  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
171375  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial precursor [KO:K02127]
171082  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial precursor [KO:K02128]
114630  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial precursor [KO:K02128]
29754  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial precursor [KO:K02128]
641434  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
192241  Atp5po; ATP synthase subunit O, mitochondrial precursor [KO:K02137]
94271  Atp5pf; ATP synthase-coupling factor 6, mitochondrial precursor [KO:K02131]
26196  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
29673  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:]
29669  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:]
29671  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:]
29674  Psma7; proteasome subunit alpha type-7 [KO:K02731] [EC:]
364814  Psma8; proteasome subunit alpha type-7-like [KO:K02731] [EC:]
29672  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:]
29668  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:]
29670  Psma3; proteasome subunit alpha type-3 [KO:K02727] [EC:]
29666  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:]
100360846  uncharacterized protein LOC100360846 [KO:K02738] [EC:]
85492  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:]
29676  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:]
29675  Psmb2; proteasome subunit beta type-2 [KO:K02734] [EC:]
29425  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:]
94198  Psmb1; proteasome subunit beta type-1 precursor [KO:K02732] [EC:]
58854  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:]
25581  Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061]
117263  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
117262  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
289990  Psmc6; 26S protease regulatory subunit 10B [KO:K03064]
29677  Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065]
81827  Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066]
287984  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
83806  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
287670  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
161475  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
287772  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
303353  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
289924  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
307821  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
365388  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
83499  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
311078  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
292766  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
65138  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
680532  Sem1; 26S proteasome complex subunit DSS1 [KO:K10881]
362119  C5; complement C5 precursor [KO:K03994]
24237  C6; complement component C6 precursor [KO:K03995]
117517  C7; LOW QUALITY PROTEIN: complement component C7 [KO:K03996]
298288  C8a; complement component C8 alpha chain precursor [KO:K03997]
313421  C8b; complement component C8 beta chain precursor [KO:K03998]
296545  C8g; complement component C8 gamma chain [KO:K03999]
117512  C9; complement component C9 [KO:K04000]
298566  C1qa; complement C1q subcomponent subunit A precursor [KO:K03986]
29687  C1qb; complement C1q subcomponent subunit B precursor [KO:K03987]
362634  C1qc; complement C1q subcomponent subunit C precursor [KO:K03988]
24468  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
24472  Hspa1b; heat shock 70 kDa protein 1B [KO:K03283]
60460  Hspa2; heat shock-related 70 kDa protein 2 [KO:K03283]
24963  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
108348108  heat shock 70 kDa protein 1A [KO:K03283]
25496  Notch1; neurogenic locus notch homolog protein 1 precursor [KO:K02599]
25404  Cav1; caveolin-1 isoform alpha [KO:K06278]
363425  Cav2; caveolin-2 [KO:K12958]
29161  Cav3; caveolin-3 [KO:K12959]
85243  Pik3cb; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:]
170911  Pik3ca; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:]
366508  Pik3cd; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922] [EC:]
25513  Pik3r1; phosphatidylinositol 3-kinase regulatory subunit alpha [KO:K02649]
60664  Pik3r3; phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649]
29741  Pik3r2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
170538  Prkcd; protein kinase C delta type [KO:K06068] [EC:]
66021  Cybb; cytochrome b-245 beta chain [KO:K21421] [EC:1.-.-.-]
79129  Cyba; cytochrome b-245 light chain [KO:K08009]
500904  Ncf4; neutrophil cytosol factor 4 [KO:K08012]
114553  Ncf1; neutrophil cytosolic factor 1 [KO:K08011]
364018  Ncf2; neutrophil cytosol factor 2 [KO:K08010]
363875  Rac1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
366957  Rac2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
81649  Mapk14; mitogen-activated protein kinase 14 [KO:K04441] [EC:]
60352  Mapk12; mitogen-activated protein kinase 12 [KO:K04441] [EC:]
29513  Mapk13; mitogen-activated protein kinase 13 [KO:K04441] [EC:]
689314  Mapk11; mitogen-activated protein kinase 11 [KO:K04441] [EC:]
25272  Mapk10; mitogen-activated protein kinase 10 isoform 2 [KO:K04440] [EC:]
50658  Mapk9; mitogen-activated protein kinase 9 isoform 2 [KO:K04440] [EC:]
116554  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:]
116590  Mapk1; mitogen-activated protein kinase 1 [KO:K04371] [EC:]
50689  Mapk3; mitogen-activated protein kinase 3 [KO:K04371] [EC:]
81646  Creb1; cyclic AMP-responsive element-binding protein 1 isoform A [KO:K05870]
81647  Atf2; cyclic AMP-dependent transcription factor ATF-2 [KO:K04450]
310616  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
362165  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
362339  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
314638  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
500131  Creb5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
406169  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
24330  Egr1; early growth response protein 1 [KO:K09203]
81780  Ccl5; C-C motif chemokine 5 precursor [KO:K12499]
24493  Il1a; interleukin-1 alpha [KO:K04383]
24494  Il1b; interleukin-1 beta [KO:K04519]
24498  Il6; interleukin-6 precursor [KO:K05405]
24835  Tnf; tumor necrosis factor [KO:K03156]
84027  Gsk3b; glycogen synthase kinase-3 beta [KO:K03083] [EC:]
116549  Csnk2a1; casein kinase II subunit alpha [KO:K03097] [EC:]
307641  Csnk2a2; casein kinase II subunit alpha' [KO:K03097] [EC:]
81650  Csnk2b; casein kinase II subunit beta [KO:K03115]
117550  Kif5b; kinesin-1 heavy chain [KO:K10396]
311024  Kif5c; kinesin heavy chain isoform 5C [KO:K10396]
314906  Kif5a; kinesin heavy chain isoform 5A [KO:K10396]
171041  Klc1; kinesin light chain 1 isoform C [KO:K10407]
171549  Klc3; kinesin light chain 3 [KO:K10407]
316226  Klc4; kinesin light chain 4 [KO:K10407]
309159  Klc2; kinesin light chain 2 [KO:K10407]
64158  Tuba1a; tubulin alpha-1A chain [KO:K07374]
291287  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
300218  Tuba1c; tubulin alpha-1C chain [KO:K07374]
316531  Tuba4a; tubulin alpha-4A chain [KO:K07374]
500319  Tuba3a; tubulin alpha-3 chain [KO:K07374]
500363  Tuba3b; tubulin alpha-3 chain [KO:K07374]
500377  Tuba8; tubulin alpha-8 chain [KO:K07374]
500929  Tuba1b; tubulin alpha-1B chain [KO:K07374]
29214  Tubb5; tubulin beta-5 chain [KO:K07375]
246118  Tubb3; tubulin beta-3 chain [KO:K07375]
291081  Tubb2b; tubulin beta-2B chain [KO:K07375]
29213  Tubb4a; tubulin beta-4A chain [KO:K07375]
307351  Tubb6; tubulin beta-6 chain [KO:K07375]
498736  Tubb2a; tubulin beta-2A chain [KO:K07375]
679312  Tubb1; tubulin beta-1 chain isoform X1 [KO:K07375]
296554  Tubb4b; tubulin beta-4B chain [KO:K07375]
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Saa P, Harris DA, Cervenakova L
Mechanisms of prion-induced neurodegeneration.
Expert Rev Mol Med 18:e5 (2016)
Goold R, McKinnon C, Tabrizi SJ
Prion degradation pathways: Potential for therapeutic intervention.
Mol Cell Neurosci 66:12-20 (2015)
Soto C, Satani N
The intricate mechanisms of neurodegeneration in prion diseases.
Trends Mol Med 17:14-24 (2011)
Mays CE, Soto C
The stress of prion disease.
Brain Res 1648:553-560 (2016)
Jones E, Mead S
Genetic risk factors for Creutzfeldt-Jakob disease.
Neurobiol Dis 142:104973 (2020)
Kovacs GG, Budka H
Prion diseases: from protein to cell pathology.
Am J Pathol 172:555-65 (2008)
Campana V, Sarnataro D, Zurzolo C
The highways and byways of prion protein trafficking.
Trends Cell Biol 15:102-11 (2005)
Caughey B, Baron GS
Prions and their partners in crime.
Nature 443:803-10 (2006)
Chiesa R, Harris DA
Prion diseases: what is the neurotoxic molecule?
Neurobiol Dis 8:743-63 (2001)
Fasano C, Campana V, Zurzolo C
Prions: protein only or something more? Overview of potential prion cofactors.
J Mol Neurosci 29:195-214 (2006)
Roucou X, Gains M, LeBlanc AC
Neuroprotective functions of prion protein.
J Neurosci Res 75:153-61 (2004)
Harris DA
Cellular biology of prion diseases.
Clin Microbiol Rev 12:429-44 (1999)
Novakofski J, Brewer MS, Mateus-Pinilla N, Killefer J, McCusker RH
Prion biology relevant to bovine spongiform encephalopathy.
J Anim Sci 83:1455-76 (2005)
Peggion C, Bertoli A, Sorgato MC
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
Biochem Biophys Res Commun 483:1148-1155 (2017)
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
Prion Protein in Glioblastoma Multiforme.
Int J Mol Sci 20:E5107 (2019)
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
Int J Mol Sci 20:E6004 (2019)
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
Mol Neurobiol 56:6035-6045 (2019)
Song Z, Zhao D, Yang L
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
Halliday M, Hughes D, Mallucci GR
Fine-tuning PERK signaling for neuroprotection.
J Neurochem 142:812-826 (2017)
Hughes D, Halliday M
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
Pathogens 6:E63 (2017)
Hetz C, Mollereau B
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
Nat Rev Neurosci 15:233-49 (2014)
Hughes D, Mallucci GR
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
FEBS J 286:342-355 (2019)
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
J Cell Mol Med 15:2025-39 (2011)
Ferreiro E, Oliveira CR, Pereira CM
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
Neurobiol Dis 30:331-42 (2008)
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
Neurobiol Dis 23:669-78 (2006)
Torres M, Encina G, Soto C, Hetz C
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
Commun Integr Biol 4:258-61 (2011)
Ciechanover A, Kwon YT
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
Exp Mol Med 47:e147 (2015)
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
Disease-associated prion protein oligomers inhibit the 26S proteasome.
Mol Cell 26:175-88 (2007)
Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
Science 284:339-43 (1999)
Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
EMBO J 22:5435-45 (2003)
Zamponi E, Pigino GF
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
Front Cell Neurosci 13:350 (2019)
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
Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
PLoS One 12:e0188340 (2017)
Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
Understanding the neurospecificity of Prion protein signaling.
Front Biosci (Landmark Ed) 16:169-86 (2011)
Didonna A
Prion protein and its role in signal transduction.
Cell Mol Biol Lett 18:209-30 (2013)
Shah SZA, Zhao D, Hussain T, Yang L
The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
Front Aging Neurosci 9:120 (2017)
Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
J Biol Chem 280:1529-34 (2005)
Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
Notch-1 activation and dendritic atrophy in prion disease.
Proc Natl Acad Sci U S A 102:886-91 (2005)
Dearmond SJ, Bajsarowicz K
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
Mol Neurodegener 5:6 (2010)
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
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases.
Mol Neurobiol 56:2159-2173 (2019)
Aguzzi A, Zhu C
Microglia in prion diseases.
J Clin Invest 127:3230-3239 (2017)
Mabbott NA
The complement system in prion diseases.
Curr Opin Immunol 16:587-93 (2004)
Mallucci G, Collinge J
Rational targeting for prion therapeutics.
Nat Rev Neurosci 6:23-34 (2005)
rno00190  Oxidative phosphorylation
rno03050  Proteasome
rno04020  Calcium signaling pathway
rno04140  Autophagy - animal
rno04141  Protein processing in endoplasmic reticulum
rno04142  Lysosome
rno04144  Endocytosis
rno04210  Apoptosis
rno04330  Notch signaling pathway
rno04610  Complement and coagulation cascades
rno04668  TNF signaling pathway
KO pathway

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