KEGG   PATHWAY: rno05020
Entry
rno05020                    Pathway                                
Name
Prion disease - Rattus norvegicus (rat)
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
rno05020  Prion disease
rno05020

Disease
H00061  Prion disease
Organism
Rattus norvegicus (rat) [GN:rno]
Gene
100188937  Cox7c; cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
100360357  Cox7cl1; cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
100360846  Psmb6l1; proteasome 20S subunit beta 6 like 1 [KO:K02738] [EC:3.4.25.1]
100361008  [KO:K02264]
100361833  Atp5mc2l1; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100361879  [KO:K02135]
100361934  Ndufb4l4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
100361960  Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271]
100362331  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100362798  Uqcr11l1; cytochrome b-c1 complex subunit 10-like [KO:K00420]
100911483  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100912357  Ndufb1; nADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
103690609  Ndufb3l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
103691107  Ndufb4l5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
103691697  Ndufb3l2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
103693740  Uqcrbl2; cytochrome b-c1 complex subunit 7-like [KO:K00417]
103694107  Ndufb3l3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
108348108  Hspa1b; heat shock 70 kDa protein 1B [KO:K03283]
114207  Ryr1; ryanodine receptor 1 [KO:K04961]
114493  Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
114553  Ncf1; neutrophil cytosolic factor 1 [KO:K08011]
114630  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial precursor [KO:K02128]
116549  Csnk2a1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
116550  Atp5f1c; ATP synthase subunit gamma, mitochondrial [KO:K02136]
116554  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
116590  Mapk1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
117036  Lamc1; laminin subunit gamma-1 precursor [KO:K05635]
117262  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
117263  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
117512  C9; complement component C9 [KO:K04000]
117517  C7; complement component C7 isoform X1 [KO:K03996]
117550  Kif5b; kinesin-1 heavy chain [KO:K10396] [EC:5.6.1.3]
120094308  [KO:K03960]
120095266  [KO:K02272]
120097699  [KO:K00420]
120098258  [KO:K02268]
120098314  [KO:K02128]
120098318  [KO:K03959]
120099907  [KO:K03946]
120099910  [KO:K08738]
120101567  [KO:K03951]
120103152  [KO:K02267]
134484121  [KO:K00922] [EC:2.7.1.153]
156117  Casp12; caspase-12 [KO:K04741] [EC:3.4.22.-]
157074  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial precursor [KO:K00234] [EC:1.3.5.1]
161475  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
170538  Prkcd; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
170546  Ryr3; ryanodine receptor 3 isoform X1 [KO:K04963]
170796  Grin3b; glutamate receptor ionotropic, NMDA 3B precursor [KO:K05214]
170911  Pik3ca; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
171041  Klc1; kinesin light chain 1 isoform C [KO:K10407]
171082  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial precursor [KO:K02128]
171335  Cox8a; cytochrome c oxidase subunit 8A, mitochondrial precursor [KO:K02273]
171374  Atp5f1b; ATP synthase subunit beta, mitochondrial precursor [KO:K02133] [EC:7.1.2.2]
171375  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial precursor [KO:K02127]
171378  Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform [KO:K04348] [EC:3.1.3.16]
171549  Klc3; kinesin light chain 3 [KO:K10407]
191573  Grin3a; glutamate receptor ionotropic, NMDA 3A isoform 1 precursor [KO:K05213]
192241  Atp5po; ATP synthase subunit O, mitochondrial precursor [KO:K02137]
192277  Stip1; stress-induced-phosphoprotein 1 [KO:K09553]
24237  C6; complement component C6 precursor [KO:K03995]
24239  Cacna1c; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
24330  Egr1; early growth response protein 1 [KO:K09203]
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]
24468  Hspa8; heat shock cognate 71 kDa protein [KO:K03283]
24472  Hspa1a; heat shock 70 kDa protein 1A [KO:K03283]
24493  Il1a; interleukin-1 alpha [KO:K04383]
24494  Il1b; interleukin-1 beta [KO:K04519]
24498  Il6; interleukin-6 precursor [KO:K05405]
24586  Ncam1; neural cell adhesion molecule 1 isoform 5 precursor [KO:K06491]
245958  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
245965  Atp5f1d; ATP synthase subunit delta, mitochondrial precursor [KO:K02134]
246118  Tubb3; tubulin beta-3 chain [KO:K07375]
24674  Ppp3ca; protein phosphatase 3 catalytic subunit alpha [KO:K04348] [EC:3.1.3.16]
24675  Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform [KO:K04348] [EC:3.1.3.16]
24686  Prnp; major prion protein precursor [KO:K05634]
24786  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
24835  Tnf; tumor necrosis factor [KO:K03156]
24887  Bax; apoptosis regulator BAX [KO:K02159]
24963  Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283]
25150  Fyn; tyrosine-protein kinase Fyn [KO:K05703] [EC:2.7.10.2]
25176  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
25250  Cox8b; cytochrome c oxidase subunit 8B, mitochondrial precursor [KO:K02273]
25262  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform 3 [KO:K04958]
25272  Mapk10; mitogen-activated protein kinase 10 isoform 2 [KO:K04440] [EC:2.7.11.24]
25278  Cox6a2; cytochrome c oxidase subunit 6A2, mitochondrial precursor [KO:K02266]
25282  Cox6a1; cytochrome c oxidase subunit 6A1, mitochondrial precursor [KO:K02266]
252934  Cox5a; cytochrome c oxidase subunit 5A, mitochondrial precursor [KO:K02264]
25309  Cycs; cytochrome c, somatic [KO:K08738]
25310  Cyct; cytochrome c, testis-specific [KO:K08738]
25402  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
25404  Cav1; caveolin-1 isoform alpha [KO:K06278]
25488  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
25496  Notch1; neurogenic locus notch homolog protein 1 precursor [KO:K02599]
25513  Pik3r1; phosphatidylinositol 3-kinase regulatory subunit alpha [KO:K02649]
25581  Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061]
25617  Hspa5; endoplasmic reticulum chaperone BiP precursor [KO:K09490] [EC:3.6.4.10]
25636  Prkaca; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
25679  Itpr3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
257648  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform 1 [KO:K04849]
26192  CYTB; cytochrome b [KO:K00412]
26193  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
26194  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
26195  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
26196  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
26197  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
26198  COX2; cytochrome c oxidase subunit II [KO:K02261]
26199  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
26200  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
26201  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
26202  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
26203  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
26204  COX3; cytochrome c oxidase subunit III [KO:K02262]
282819  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial precursor [KO:K09565] [EC:5.2.1.8]
287670  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
287772  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
287984  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
288088  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
288280  Ncam2; neural cell adhesion molecule 2 precursor [KO:K06491]
289217  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
289218  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial precursor [KO:K03935] [EC:7.1.1.2]
289924  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
289990  Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064]
290019  Cycsl3; cytochrome c, somatic-like [KO:K08738]
291081  Tubb2b; tubulin beta-2B chain [KO:K07375]
291103  Uqcrfs1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
291287  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
29161  Cav3; caveolin-3 [KO:K12959]
291660  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
29213  Tubb4a; tubulin beta-4A chain [KO:K07375]
29214  Tubb5; tubulin beta-5 chain [KO:K07375]
292766  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
293130  Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
293448  Uqcrc2; cytochrome b-c1 complex subunit 2, mitochondrial precursor [KO:K00415]
293453  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
293508  Prkacb; cAMP-dependent protein kinase catalytic subunit beta [KO:K04345] [EC:2.7.11.11]
293652  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
293655  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
293991  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
29425  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
29445  Cox4i1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial precursor [KO:K02263]
294560  Mcu; calcium uniporter protein, mitochondrial precursor [KO:K20858]
29467  Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452]
294964  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
29507  Cox7a2; cytochrome c oxidase subunit 7A2, mitochondrial precursor [KO:K02270]
29513  Mapk13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
295923  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
296169  Psmb3l2; proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
296545  C8g; complement component C8 gamma chain isoform 1 precursor [KO:K03999]
296554  Tubb4b; tubulin beta-4B chain [KO:K07375]
296658  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
29666  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
29668  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
29669  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
29670  Psma3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
29671  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
29672  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
29673  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
29674  Psma7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
29675  Psmb2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
29676  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
29677  Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065]
29687  C1qb; complement C1q subcomponent subunit B precursor [KO:K03987]
29702  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 precursor [KO:K08860] [EC:2.7.11.1]
29716  Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851]
29741  Pik3r2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
29748  Ppp3r1; calcineurin subunit B type 1 [KO:K06268]
29749  Ppp3r2; calcineurin subunit B type 2 [KO:K06268]
29754  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial precursor [KO:K02128]
297990  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
298288  C8a; complement component C8 alpha chain precursor [KO:K03997]
298400  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
298566  C1qa; complement C1q subcomponent subunit A precursor [KO:K03986]
298596  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235] [EC:1.3.5.1]
298762  Cox7a2l; cytochrome c oxidase subunit 7A2 like isoform 1 [KO:K02270]
299310  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
299643  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
299739  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
299954  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
300047  Cyc1; cytochrome c-1 [KO:K00413]
300218  Tuba1c; tubulin alpha-1C chain [KO:K07374]
301011  Uqcrc1; cytochrome b-c1 complex subunit 1, mitochondrial precursor [KO:K00414]
301123  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
301427  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
301458  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial precursor [KO:K03934] [EC:7.1.1.2]
303353  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
303393  Cox7b; cytochrome c oxidase subunit 7B, mitochondrial precursor [KO:K02271]
307351  Tubb6; tubulin beta-6 chain [KO:K07375]
307641  Csnk2a2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
307821  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
309159  Klc2; kinesin light chain 2 [KO:K10407]
310616  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform 1 [KO:K09048]
311024  Kif5c; kinesin heavy chain isoform 5C [KO:K10396] [EC:5.6.1.3]
311078  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
313421  C8b; complement component C8 beta chain precursor [KO:K03998]
314638  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
314906  Kif5a; kinesin heavy chain isoform 5A [KO:K10396] [EC:5.6.1.3]
315167  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
316226  Klc4; kinesin light chain 4 [KO:K10407]
316531  Tuba4a; tubulin alpha-4A chain [KO:K07374]
316632  Ndufa10l1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10-like [KO:K03954]
360229  Cox8c; cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
361385  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
362119  C5; complement C5 precursor [KO:K03994]
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]
362344  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
362440  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
362588  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
362634  C1qc; complement C1q subcomponent subunit C precursor [KO:K03988]
362837  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
362897  Uqcrb; cytochrome b-c1 complex subunit 7 [KO:K00417]
363061  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
363425  Cav2; caveolin-2 [KO:K12958]
363441  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
363875  Rac1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
364018  Ncf2; neutrophil cytosol factor 2 [KO:K08010]
364814  Psma8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
365388  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
366448  Uqcrh; cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
366508  Pik3cd; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922] [EC:2.7.1.153]
366614  Atp5mc1l2; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
366957  Rac2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
367149  Bax-ps1; apoptosis regulator BAX-like [KO:K02159]
406169  Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
497902  Uqcrq; cytochrome b-c1 complex subunit 8 [KO:K00418]
498736  Tubb2a; tubulin beta-2A chain [KO:K07375]
498885  Slc25a6; solute carrier family 25 member 6 [KO:K05863]
499131  Ndufb4l3; similar to NADH:ubiquinone oxidoreductase B15 subunit [KO:K03960]
499512  Atp5mc2l2; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
499529  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial precursor [KO:K03937]
500131  Creb5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
500319  Tuba3a; tubulin alpha-3 chain [KO:K07374]
500363  Tuba3b; tubulin alpha-3 chain [KO:K07374]
500377  Tuba8; tubulin alpha-8 chain [KO:K07374]
500871  Slc25a5l1; ADP/ATP translocase 2-like [KO:K05863]
500904  Ncf4; neutrophil cytosol factor 4 [KO:K08012]
500929  Tuba1b; tubulin alpha-1B chain [KO:K07374]
501886  Ndufb4l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
50658  Mapk9; mitogen-activated protein kinase 9 isoform 2 [KO:K04440] [EC:2.7.11.24]
50689  Mapk3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
54318  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
54322  Cox6c; cytochrome c oxidase subunit 6C-2 [KO:K02268]
58854  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
58918  Casp9; caspase-9 [KO:K04399] [EC:3.4.22.62]
60352  Mapk12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
60664  Pik3r3; phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649]
641434  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
64158  Tuba1a; tubulin alpha-1A chain [KO:K07374]
64539  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform 2 [KO:K03944]
64639  Bad; bcl2-associated agonist of cell death [KO:K02158]
65138  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
65262  Atp5f1a; ATP synthase subunit alpha, mitochondrial precursor [KO:K02132]
654441  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
66021  Cybb; cytochrome b-245 beta chain [KO:K21421] [EC:1.-.-.-]
678759  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial precursor [KO:K03954]
679312  Tubb1; tubulin beta-1 chain isoform X1 [KO:K07375]
679739  Ndufs6; NADH:ubiquinone oxidoreductase subunit S6-like 1 [KO:K03939]
679794  Cycsl1; cytochrome c, somatic-like [KO:K08738]
680121  Haus8l2; heat shock cognate 71 kDa protein-like [KO:K03283]
680532  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
681024  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
681314  [KO:K02159]
681418  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
682930  Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
685322  Uqcr10; ubiquinol-cytochrome c reductase complex 7.2kDa protein [KO:K00419]
685596  Uqcrbl1; cytochrome b-c1 complex subunit 7-like [KO:K00417]
687508  Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270]
688022  Atp5pdl1; ATP synthase subunit d, mitochondrial-like [KO:K02138]
688386  Cox7a2l2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
688869  Cox6b1; cytochrome c oxidase subunit 6B1 [KO:K02267]
689108  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
689314  Mapk11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
689560  Ryr2; ryanodine receptor 2 isoform 1 [KO:K04962]
689938  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
689961  Atp5mc1l1; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
690675  Cycsl2; cytochrome c, somatic-like [KO:K08738]
690848  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
691001  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
78963  Apaf1; apoptotic protease-activating factor 1 [KO:K02084]
79129  Cyba; cytochrome b-245 light chain [KO:K08009]
79255  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
81646  Creb1; cyclic AMP-responsive element-binding protein 1 isoform A [KO:K05870]
81647  Atf2; cyclic AMP-dependent transcription factor ATF-2 [KO:K04450]
81649  Mapk14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
81650  Csnk2b; casein kinase II subunit beta [KO:K03115]
81678  Itpr2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
81728  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial precursor [KO:K03943] [EC:7.1.1.2]
81780  Ccl5; C-C motif chemokine 5 precursor [KO:K12499]
81827  Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066]
83499  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
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]
83806  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
84027  Gsk3b; glycogen synthase kinase-3 beta [KO:K03083] [EC:2.7.11.26]
84683  Cox4i2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform 2 precursor [KO:K02263]
85243  Pik3cb; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
85333  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
85492  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
94194  Cox5b; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265]
94198  Psmb1; proteasome subunit beta type-1 precursor [KO:K02732] [EC:3.4.25.1]
94271  Atp5pf; ATP synthase-coupling factor 6, mitochondrial precursor [KO:K02131]
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)
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Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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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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Reference
PMID:32565065
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Neurobiol Dis 142:104973 (2020)
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Reference
PMID:18245809
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Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
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Am J Pathol 172:555-65 (2008)
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Reference
PMID:15695097
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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)
DOI:10.1016/j.tcb.2004.12.002
Reference
PMID:17051207
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
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Nature 443:803-10 (2006)
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Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
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Reference
PMID:17085779
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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)
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Reference
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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Reference
PMID:10398674
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Harris DA
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Cellular biology of prion diseases.
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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.
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Biochem Biophys Res Commun 483:1148-1155 (2017)
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Reference
PMID:31618844
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
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Int J Mol Sci 20:E5107 (2019)
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Reference
PMID:31795242
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
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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
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
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Mol Neurobiol 56:6035-6045 (2019)
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Reference
PMID:23439666
  Authors
Song Z, Zhao D, Yang L
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Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
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Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
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Reference
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  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
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J Neurochem 142:812-826 (2017)
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Reference
PMID:29194372
  Authors
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  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
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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Reference
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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
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Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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J Cell Mol Med 15:2025-39 (2011)
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Reference
PMID:18420416
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
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Neurobiol Dis 30:331-42 (2008)
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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)
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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)
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Reference
PMID:25766616
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Ciechanover A, Kwon YT
  Title
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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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)
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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
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
ko05020   

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