KEGG   PATHWAY: mcf05020
Entry
mcf05020                    Pathway                                
Name
Prion disease - Macaca fascicularis (crab-eating macaque)
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
mcf05020  Prion disease
mcf05020

Disease
H00061  Prion disease
Organism
Macaca fascicularis (crab-eating macaque) [GN:mcf]
Gene
101864886  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
101864983  VDAC1; non-selective voltage-gated ion channel VDAC1 [KO:K05862]
101865051  PSMC5; 26S proteasome regulatory subunit 8 isoform X2 [KO:K03066]
101865119  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101865175  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X4 [KO:K04850]
101865238  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
101865240  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
101865389  ITPR1; inositol 1,4,5-trisphosphate-gated calcium channel ITPR1 isoform X2 [KO:K04958]
101865454  KIF5A; kinesin heavy chain [KO:K10396] [EC:5.6.1.3]
101865478  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
101865498  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
101865560  CSNK2A2; casein kinase II subunit alpha' isoform X2 [KO:K03097] [EC:2.7.11.1]
101865627  VDAC2; non-selective voltage-gated ion channel VDAC2 isoform X1 [KO:K15040]
101865646  CAV1; caveolin-1 isoform X2 [KO:K06278]
101865772  [KO:K07374]
101865806  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
101865843  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
101865852  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
101865964  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
101866070  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
101866246  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X2 [KO:K02649]
101866339  KLC2; kinesin light chain 2 [KO:K10407]
101866438  TUBB4B; tubulin beta-4B chain [KO:K07375]
101866439  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
101866581  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
101866593  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X2 [KO:K04392]
101866603  NCAM2; neural cell adhesion molecule 2 isoform X3 [KO:K06491]
101866604  [KO:K07375]
101866612  PSMA8; proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
101866670  NDUFAB1; acyl carrier protein, mitochondrial isoform X1 [KO:K03955]
101866673  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
101866792  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X11 [KO:K03941] [EC:7.1.1.2]
101866824  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X2 [KO:K03961]
101866879  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101866963  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
101867105  TUBA8; tubulin alpha-8 chain isoform X2 [KO:K07374]
101867140  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
101867279  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
101867305  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
101867352  MAPK13; mitogen-activated protein kinase 13 isoform X2 [KO:K04441] [EC:2.7.11.24]
101867500  TUBA1B; tubulin alpha-1B chain [KO:K07374]
101925039  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
101925169  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101925194  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
101925240  [KO:K03283]
101925386  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
101925452  TUBA3E; tubulin alpha-3E chain [KO:K07374]
101925455  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
101925535  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
101925588  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
101925689  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
101925816  TUBB; tubulin beta chain isoform X1 [KO:K07375]
101925833  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
101925838  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
101925928  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
101925940  TUBB3; tubulin beta-3 chain [KO:K07375]
101926012  HSPA1L; heat shock 70 kDa protein 1-like isoform X2 [KO:K03283]
101926075  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
101926377  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
101926424  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
101926461  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X3 [KO:K04345] [EC:2.7.11.11]
101926508  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
101926546  IL1A; interleukin-1 alpha isoform X2 [KO:K04383]
101926612  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
101926640  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101926738  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
101926770  MAPK14; mitogen-activated protein kinase 14 isoform X3 [KO:K04441] [EC:2.7.11.24]
101926805  TUBA4A; tubulin alpha-4A chain isoform X1 [KO:K07374]
102114833  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
102114855  [KO:K07375]
102114911  NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
102115033  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X2 [KO:K05208]
102115672  ATP5PB; ATP synthase peripheral stalk subunit b, mitochondrial isoform X3 [KO:K02127]
102115744  ITPR2; inositol 1,4,5-trisphosphate-gated calcium channel ITPR2 isoform X1 [KO:K04959]
102115795  [KO:K04741] [EC:3.4.22.-]
102115854  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
102116003  ITPR3; inositol 1,4,5-trisphosphate-gated calcium channel ITPR3 isoform X1 [KO:K04960]
102116032  C6; complement component C6 [KO:K03995]
102116045  LAMC1; laminin subunit gamma-1 isoform X1 [KO:K05635]
102116211  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
102116447  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
102116497  COX6A2; cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
102116568  VDAC3; non-selective voltage-gated ion channel VDAC3 isoform X1 [KO:K15041]
102116573  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
102116683  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
102116918  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
102116996  MAPK3; mitogen-activated protein kinase 3 isoform X1 [KO:K04371] [EC:2.7.11.24]
102117050  [KO:K03935] [EC:7.1.1.2]
102117102  [KO:K08738]
102117222  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
102117303  UQCRB; cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
102117377  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
102117414  PRKACG; cAMP-dependent protein kinase catalytic subunit gamma [KO:K04345] [EC:2.7.11.11]
102117433  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
102117567  ATP5PF; ATP synthase peripheral stalk subunit F6, mitochondrial isoform X2 [KO:K02131]
102117938  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
102118472  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X3 [KO:K03954]
102118497  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
102118608  COX7A2L; cytochrome c oxidase subunit 7A2-like, mitochondrial isoform X2 [KO:K02270]
102118619  [KO:K02265]
102118687  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
102118944  TUBB1; tubulin beta-1 chain [KO:K07375]
102119049  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X4 [KO:K03940] [EC:7.1.1.2]
102119570  ATP5F1E; ATP synthase F(1) complex subunit epsilon, mitochondrial [KO:K02135]
102119749  IL1B; interleukin-1 beta [KO:K04519]
102119841  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
102120205  CYCS; cytochrome c [KO:K08738]
102120762  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
102121218  [KO:K07374]
102121272  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
102121361  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128]
102121560  [KO:K02267]
102121921  BAD; bcl2-associated agonist of cell death [KO:K02158]
102122005  CAV2; caveolin-2 isoform X1 [KO:K12958]
102122063  COX8C; cytochrome c oxidase subunit 8C, mitochondrial isoform X3 [KO:K02273]
102122149  [KO:K02128]
102122399  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X5 [KO:K03947]
102122418  CYBB; NADPH oxidase 2 [KO:K21421] [EC:1.-.-.-]
102122521  [KO:K03943] [EC:7.1.1.2]
102122711  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
102122725  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
102122883  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
102122953  PSMC6; 26S proteasome regulatory subunit 10B isoform X2 [KO:K03064]
102123032  [KO:K00416]
102123100  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
102123126  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
102123175  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
102123270  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
102123367  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
102123745  ATP5F1A; ATP synthase F(1) complex subunit alpha, mitochondrial [KO:K02132]
102123783  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 isoform X1 [KO:K03038]
102124180  COX7B; cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
102124346  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
102124562  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
102124626  [KO:K02265]
102124686  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X15 [KO:K03943] [EC:7.1.1.2]
102124773  PSMB3; proteasome subunit beta type-3 isoform X1 [KO:K02735] [EC:3.4.25.1]
102124789  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X2 [KO:K09565] [EC:5.2.1.8]
102124804  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
102124843  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
102124883  [KO:K03064]
102125060  [KO:K07375]
102125227  CSNK2B; casein kinase II subunit beta isoform X1 [KO:K03115]
102125333  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X2 [KO:K03953]
102125658  C5; complement C5 isoform X1 [KO:K03994]
102125825  CSNK2A1; casein kinase II subunit alpha isoform X2 [KO:K03097] [EC:2.7.11.1]
102126003  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X2 [KO:K00234] [EC:1.3.5.1]
102126339  MAPK8; mitogen-activated protein kinase 8 isoform X2 [KO:K04440] [EC:2.7.11.24]
102126517  COX7A2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
102126571  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
102126692  C8G; complement component C8 gamma chain [KO:K03999]
102126830  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
102126878  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
102127185  [KO:K00236]
102127229  PRNP; major prion protein [KO:K05634]
102127470  ATP5PO; ATP synthase peripheral stalk subunit OSCP, mitochondrial [KO:K02137]
102127531  RYR3; ryanodine receptor 3 isoform X1 [KO:K04963]
102127614  [KO:K08738]
102127751  [KO:K00236]
102127986  GRIN3B; glutamate receptor ionotropic, NMDA 3B isoform X1 [KO:K05214]
102128230  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
102128326  EGR1; early growth response protein 1 [KO:K09203]
102128569  KIF5C; kinesin heavy chain [KO:K10396] [EC:5.6.1.3]
102128659  UQCR11; cytochrome b-c1 complex subunit 10 [KO:K00420]
102128802  [KO:K07374]
102129176  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
102129216  [KO:K02127]
102129286  TUBB4A; tubulin beta-4A chain [KO:K07375]
102129446  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
102129485  [KO:K02128]
102129553  RYR1; ryanodine receptor 1 isoform X1 [KO:K04961]
102129733  COX7C; cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
102129785  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
102129825  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
102130095  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
102130191  COX6B2; cytochrome c oxidase subunit 6B2 [KO:K02267]
102130549  C1QC; complement C1q subcomponent subunit C isoform X1 [KO:K03988]
102130865  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
102131065  [KO:K07375]
102131294  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
102131553  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857]
102131632  DDIT3; DNA damage-inducible transcript 3 protein isoform X2 [KO:K04452]
102132017  [KO:K00411] [EC:7.1.1.8]
102132064  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
102132250  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
102132255  BAX; apoptosis regulator BAX isoform X7 [KO:K02159]
102132307  RYR2; ryanodine receptor 2 isoform X5 [KO:K04962]
102132705  [KO:K03949]
102132790  COX5B; cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
102133057  UQCRQ; cytochrome b-c1 complex subunit 8 [KO:K00418]
102133224  ATP5F1C; ATP synthase F(1) complex subunit gamma, mitochondrial isoform X2 [KO:K02136]
102133278  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
102133338  C8B; complement component C8 beta chain isoform X1 [KO:K03998]
102133526  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
102133631  UQCRFS1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
102133722  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
102133753  CAV3; caveolin-3 [KO:K12959]
102133756  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
102133949  UQCR10; cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
102134019  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
102134095  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X2 [KO:K09048]
102134282  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
102134309  [KO:K00236]
102134421  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
102134477  PPP3CA; protein phosphatase 3 catalytic subunit alpha isoform X1 [KO:K04348] [EC:3.1.3.16]
102134986  [KO:K03955]
102135041  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
102135458  STIP1; stress-induced-phosphoprotein 1 isoform X1 [KO:K09553]
102135711  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
102135755  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
102135848  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
102135950  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
102136158  PRKCD; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
102136267  UQCRH; cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
102136437  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
102137056  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
102137128  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X3 [KO:K03937]
102137250  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
102137291  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
102137297  COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
102137340  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
102137362  [KO:K02272]
102137382  C9; complement component C9 [KO:K04000]
102137473  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
102137566  [KO:K07375]
102137573  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
102137623  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
102137931  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
102138179  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
102138213  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X10 [KO:K04851]
102138307  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
102138538  [KO:K02263]
102138616  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
102138876  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
102138971  IL6; interleukin-6 precursor [KO:K05405]
102139017  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
102139269  [KO:K07375]
102139434  C1QB; complement C1q subcomponent subunit B [KO:K03987]
102139510  COX8A; cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
102139631  TNF; tumor necrosis factor [KO:K03156]
102139679  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
102139855  COX4I2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
102140105  ATP5F1B; ATP synthase F(1) complex subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
102140196  KIF5B; kinesin-1 heavy chain [KO:K10396] [EC:5.6.1.3]
102140374  [KO:K11351]
102140826  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
102141324  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
102141417  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
102141433  COX6A1; cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
102142021  ATP5PD; ATP synthase peripheral stalk subunit d, mitochondrial isoform X2 [KO:K02138]
102142026  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
102142308  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
102142378  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
102142443  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
102142948  [KO:K03949]
102142969  GRIN2D; glutamate receptor ionotropic, NMDA 2D isoform X2 [KO:K05212]
102143103  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X8 [KO:K06691]
102143176  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
102143409  [KO:K03938]
102143464  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
102143674  SLC25A31; ADP/ATP translocase 4 isoform X2 [KO:K05863]
102143747  NCF4; neutrophil cytosol factor 4 isoform X1 [KO:K08012]
102143756  COX6B1; cytochrome c oxidase subunit 6B1 [KO:K02267]
102143777  CYBA; cytochrome b-245 light chain isoform X5 [KO:K08009]
102144182  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
102144192  NCAM1; neural cell adhesion molecule 1 isoform X3 [KO:K06491]
102144280  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X2 [KO:K02263]
102144739  ATP5F1D; ATP synthase F(1) complex subunit delta, mitochondrial isoform X1 [KO:K02134]
102144790  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
102145011  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
102145434  [KO:K03947]
102145737  CYC1; cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
102145957  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X16 [KO:K00236]
102145991  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
102146369  [KO:K03938]
102146405  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
102146522  [KO:K03965]
102147055  C7; complement component C7 [KO:K03996]
102147252  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
107126479  C1QA; complement C1q subcomponent subunit A [KO:K03986]
107127795  CCL5; C-C motif chemokine 5 [KO:K12499]
107127908  [KO:K08738]
107129296  [KO:K03062]
107130218  [KO:K08738]
123567561  [KO:K02272]
123568376  [KO:K02272]
123568388  [KO:K03959]
123569396  [KO:K07375]
123570066  [KO:K00237]
123570224  [KO:K02272]
123571146  [KO:K02270]
123572509  [KO:K02272]
123572795  [KO:K03283]
123572831  [KO:K02272]
123573614  [KO:K02268]
123573956  [KO:K02272]
123574038  [KO:K03948]
123574408  [KO:K02272]
123574442  [KO:K02272]
123575162  [KO:K02272]
123575527  [KO:K03948]
135964855  [KO:K07375]
135966624  [KO:K00237]
135969579  [KO:K07375]
135970856  [KO:K07375]
141406880  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
141409493  [KO:K05863]
7857739  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
7857740  COX2; cytochrome c oxidase subunit II [KO:K02261]
7857741  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
7857742  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
7857743  COX3; cytochrome c oxidase subunit III [KO:K02262]
7857744  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
7857745  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
7857746  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
7857747  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
7857748  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
7857749  CYTB; cytochrome b [KO:K00412]
7857750  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
7857751  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
  Authors
Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
DOI:10.1017/erm.2016.8
Reference
  Authors
Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
  Journal
Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
Reference
  Authors
Soto C, Satani N
  Title
The intricate mechanisms of neurodegeneration in prion diseases.
  Journal
Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
  Authors
Mays CE, Soto C
  Title
The stress of prion disease.
  Journal
Brain Res 1648:553-560 (2016)
DOI:10.1016/j.brainres.2016.04.009
Reference
  Authors
Jones E, Mead S
  Title
Genetic risk factors for Creutzfeldt-Jakob disease.
  Journal
Neurobiol Dis 142:104973 (2020)
DOI:10.1016/j.nbd.2020.104973
Reference
  Authors
Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
  Journal
Am J Pathol 172:555-65 (2008)
DOI:10.2353/ajpath.2008.070442
Reference
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
  Journal
Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
  Journal
Nature 443:803-10 (2006)
DOI:10.1038/nature05294
Reference
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
  Journal
Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
  Authors
Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
  Journal
J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
  Authors
Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
  Journal
J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
  Journal
Clin Microbiol Rev 12:429-44 (1999)
Reference
  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
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
  Journal
Biochem Biophys Res Commun 483:1148-1155 (2017)
DOI:10.1016/j.bbrc.2016.07.118
Reference
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
  Journal
Int J Mol Sci 20:E5107 (2019)
DOI:10.3390/ijms20205107
Reference
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
  Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
  Journal
Int J Mol Sci 20:E6004 (2019)
DOI:10.3390/ijms20236004
Reference
  Authors
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
  Journal
Mol Neurobiol 56:6035-6045 (2019)
DOI:10.1007/s12035-019-1505-6
Reference
  Authors
Song Z, Zhao D, Yang L
  Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
  Journal
Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
  Journal
Pathogens 6:E63 (2017)
DOI:10.3390/pathogens6040063
Reference
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
  Journal
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
Reference
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
  Journal
Neurobiol Dis 30:331-42 (2008)
DOI:10.1016/j.nbd.2008.02.003
Reference
  Authors
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
  Title
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
  Journal
Neurobiol Dis 23:669-78 (2006)
DOI:10.1016/j.nbd.2006.05.011
Reference
  Authors
Torres M, Encina G, Soto C, Hetz C
  Title
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
  Journal
Commun Integr Biol 4:258-61 (2011)
DOI:10.4161/cib.4.3.15019
Reference
  Authors
Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
  Journal
Exp Mol Med 47:e147 (2015)
DOI:10.1038/emm.2014.117
Reference
  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
  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
  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
  Authors
Zamponi E, Pigino GF
  Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
  Journal
Front Cell Neurosci 13:350 (2019)
DOI:10.3389/fncel.2019.00350
Reference
  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
  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
  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
  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
  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
  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
  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
  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
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
  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
  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
mcf00190  Oxidative phosphorylation
mcf03050  Proteasome
mcf04020  Calcium signaling pathway
mcf04140  Autophagy - animal
mcf04141  Protein processing in endoplasmic reticulum
mcf04142  Lysosome
mcf04144  Endocytosis
mcf04210  Apoptosis
mcf04330  Notch signaling pathway
mcf04610  Complement and coagulation cascades
mcf04668  TNF signaling pathway
KO pathway
ko05020   

DBGET integrated database retrieval system