KEGG   PATHWAY: csab05020
Entry
csab05020                   Pathway                                
Name
Prion disease - Chlorocebus sabaeus (green monkey)
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
csab05020  Prion disease
csab05020

Disease
H00061  Prion disease
Organism
Chlorocebus sabaeus (green monkey) [GN:csab]
Gene
103214808  [KO:K02128]
103214941  NDUFS6; NADH:ubiquinone oxidoreductase subunit S6 [KO:K03939]
103215165  C9; complement C9 [KO:K04000]
103215183  C7; complement C7 [KO:K03996]
103215186  C6; complement C6 [KO:K03995]
103215231  SDHA; succinate dehydrogenase complex flavoprotein subunit A [KO:K00234] [EC:1.3.5.1]
103215235  [KO:K00234] [EC:1.3.5.1]
103215459  PPP3CC; protein phosphatase 3 catalytic subunit gamma [KO:K04348] [EC:3.1.3.16]
103215691  VDAC3; voltage dependent anion channel 3 [KO:K15041]
103215835  NDUFS8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2]
103215844  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
103215933  PPIF; peptidylprolyl isomerase F [KO:K09565] [EC:5.2.1.8]
103215964  VDAC2; voltage dependent anion channel 2 [KO:K15040]
103215997  PPP3CB; protein phosphatase 3 catalytic subunit beta [KO:K04348] [EC:3.1.3.16]
103216000  CSNK2A1; casein kinase 2 alpha 1 [KO:K03097] [EC:2.7.11.1]
103216021  MCU; mitochondrial calcium uniporter [KO:K20858]
103216390  NDUFB8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
103216555  NDUFV1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2]
103216644  ATP5O; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137]
103216844  TUBA3E; tubulin alpha 3e [KO:K07374]
103216985  [KO:K00236]
103217064  KIF5C; kinesin family member 5C [KO:K10396]
103217172  SOD1; superoxide dismutase 1 [KO:K04565] [EC:1.15.1.1]
103217175  PSMD14; proteasome 26S subunit, non-ATPase 14 [KO:K03030]
103217363  ATF2; activating transcription factor 2 [KO:K04450]
103217364  ATP5G3; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C3 (subunit 9) [KO:K02128]
103217629  NDUFB3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
103217702  NDUFS1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2]
103217717  CREB1; cAMP responsive element binding protein 1 [KO:K05870]
103217773  ATP5J; ATP synthase, H+ transporting, mitochondrial Fo complex subunit F6 [KO:K02131]
103217904  TUBA4A; tubulin alpha 4a [KO:K07374]
103218029  NCAM2; neural cell adhesion molecule 2 [KO:K06491]
103218204  NDUFA10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
103218332  PSMD1; proteasome 26S subunit, non-ATPase 1 [KO:K03032]
103218385  CACNA1C; calcium voltage-gated channel subunit alpha1 C [KO:K04850]
103218424  NDUFA9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
103218664  GRIN2B; glutamate ionotropic receptor NMDA type subunit 2B [KO:K05210]
103218776  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
103218895  NDUFA8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
103218914  C5; complement C5 [KO:K03994]
103218927  [KO:K00416]
103219106  PPP3R2; protein phosphatase 3 regulatory subunit B, beta [KO:K06268]
103219109  GRIN3A; glutamate ionotropic receptor NMDA type subunit 3A [KO:K05213]
103219209  CREB3; cAMP responsive element binding protein 3 [KO:K09048]
103219306  NDUFB6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
103219478  NDUFV3; NADH:ubiquinone oxidoreductase subunit V3 [KO:K03944]
103219549  PRKACG; protein kinase cAMP-activated catalytic subunit gamma [KO:K04345] [EC:2.7.11.11]
103219556  [KO:K08738]
103219870  [KO:K03949]
103220127  PPP3R1; protein phosphatase 3 regulatory subunit B, alpha [KO:K06268]
103220405  COX7A2L; cytochrome c oxidase subunit 7A2 like [KO:K02270]
103220507  [KO:K05863]
103221134  PSMD2; proteasome 26S subunit, non-ATPase 2 [KO:K03028]
103221202  NDUFB5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
103221212  PIK3CA; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha [KO:K00922] [EC:2.7.1.153]
103221350  NDUFS4; NADH:ubiquinone oxidoreductase subunit S4 [KO:K03937]
103221478  KLC4; kinesin light chain 4 [KO:K10407]
103221595  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
103221596  MAPK14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
103221654  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
103221718  ATF6B; activating transcription factor 6 beta [KO:K09049]
103221743  HSPA1B; heat shock protein family A (Hsp70) member 1B [KO:K03283]
103221744  HSPA1L; heat shock protein family A (Hsp70) member 1 like [KO:K03283]
103221745  HSPA1A; heat shock protein family A (Hsp70) member 1A [KO:K03283]
103221761  CSNK2B; casein kinase 2 beta [KO:K03115]
103221819  TUBB; tubulin beta class I [KO:K07375]
103222274  [KO:K07375]
103222280  [KO:K07375]
103222477  ATP5A1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132]
103222594  PIK3R1; phosphoinositide-3-kinase regulatory subunit 1 [KO:K02649]
103222611  PSMA8; proteasome subunit alpha 8 [KO:K02731] [EC:3.4.25.1]
103222752  NDUFV2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2]
103222808  TUBB6; tubulin beta 6 class V [KO:K07375]
103222884  TUBA8; tubulin alpha 8 [KO:K07374]
103222941  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
103223148  UQCR10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
103223257  NCF4; neutrophil cytosolic factor 4 [KO:K08012]
103223272  RAC2; ras-related C3 botulinum toxin substrate 2 (rho family, small GTP binding protein Rac2) [KO:K07860]
103223345  ATF4; activating transcription factor 4 [KO:K04374]
103223414  NDUFA6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
103223551  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
103223553  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
103223677  HSPA6; heat shock protein family A (Hsp70) member 6 [KO:K03283]
103223678  SDHC; succinate dehydrogenase complex subunit C [KO:K00236]
103223688  NDUFS2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2]
103223878  [KO:K03038]
103223942  CREB3L4; cAMP responsive element binding protein 3 like 4 [KO:K09048]
103224033  PSMB4; proteasome subunit beta 4 [KO:K02736] [EC:3.4.25.1]
103224043  PSMD4; proteasome 26S subunit, non-ATPase 4 [KO:K03029]
103224193  KLC2; kinesin light chain 2 [KO:K10407]
103224226  ATP5F1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit B1 [KO:K02127]
103224293  PSMA5; proteasome subunit alpha 5 [KO:K02729] [EC:3.4.25.1]
103224540  PRKACB; protein kinase cAMP-activated catalytic subunit beta [KO:K04345] [EC:2.7.11.11]
103224737  C8B; complement C8 beta chain [KO:K03998]
103224738  C8A; complement C8 alpha chain [KO:K03997]
103224843  [KO:K08738]
103224893  UQCRH; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
103224900  PIK3R3; phosphoinositide-3-kinase regulatory subunit 3 [KO:K02649]
103225005  [KO:K00236]
103225129  PSMB2; proteasome subunit beta 2 [KO:K02734] [EC:3.4.25.1]
103225256  [KO:K03950]
103225408  C1QB; complement C1q B chain [KO:K03987]
103225409  C1QC; complement C1q C chain [KO:K03988]
103225410  C1QA; complement C1q A chain [KO:K03986]
103225507  SDHB; succinate dehydrogenase complex iron sulfur subunit B [KO:K00235] [EC:1.3.5.1]
103225550  CASP9; caspase 9 [KO:K04399] [EC:3.4.22.62]
103225637  PIK3CD; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit delta [KO:K00922] [EC:2.7.1.153]
103225665  [KO:K08738]
103226162  PSMA2; proteasome subunit alpha 2 [KO:K02726] [EC:3.4.25.1]
103226299  CREB5; cAMP responsive element binding protein 5 [KO:K09047]
103226390  IL6; interleukin 6 [KO:K05405]
103226429  NDUFB10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
103226480  NDUFA4; NDUFA4, mitochondrial complex associated [KO:K03948]
103226516  SEM1; SEM1, 26S proteasome complex subunit [KO:K10881]
103226684  PSMC2; proteasome 26S subunit, ATPase 2 [KO:K03061]
103226769  CAV2; caveolin 2 [KO:K12958]
103226770  CAV1; caveolin 1 [KO:K06278]
103226813  NDUFA5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
103226974  CREB3L2; cAMP responsive element binding protein 3 like 2 [KO:K09048]
103227023  NDUFB2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
103227597  UQCRC1; ubiquinol-cytochrome c reductase core protein I [KO:K00414]
103227755  PRKCD; protein kinase C delta [KO:K06068] [EC:2.7.11.13]
103227762  CACNA1D; calcium voltage-gated channel subunit alpha1 D [KO:K04851]
103227837  PSMD6; proteasome 26S subunit, non-ATPase 6 [KO:K03037]
103227941  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
103227961  CAV3; caveolin 3 [KO:K12959]
103228018  [KO:K08738]
103228072  GRIN2A; glutamate ionotropic receptor NMDA type subunit 2A [KO:K05209]
103228104  [KO:K03960]
103228205  NDUFB4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
103228213  GSK3B; glycogen synthase kinase 3 beta [KO:K03083] [EC:2.7.11.26]
103228810  [KO:K03938]
103228819  PSMA6; proteasome subunit alpha 6 [KO:K02730] [EC:3.4.25.1]
103228995  PSMC6; proteasome 26S subunit, ATPase 6 [KO:K03064]
103229087  PSMA3; proteasome subunit alpha 3 [KO:K02727] [EC:3.4.25.1]
103229205  EIF2S1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
103229536  PSMC1; proteasome 26S subunit, ATPase 1 [KO:K03062]
103229564  UQCR2; ubiquinol-cytochrome c reductase core protein II [KO:K00415]
103229567  NDUFB1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
103229590  COX8C; cytochrome c oxidase subunit 8C [KO:K02273]
103229745  NDUFAB1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
103229927  [KO:K10407]
103230339  CACNA1S; calcium voltage-gated channel subunit alpha1 S [KO:K04857]
103230452  NCF2; neutrophil cytosolic factor 2 [KO:K08010]
103230459  LAMC1; laminin subunit gamma 1 [KO:K05635]
103230532  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
103230821  RYR2; ryanodine receptor 2 [KO:K04962]
103230925  [KO:K03283]
103231423  COX6A2; cytochrome c oxidase subunit 6A2 [KO:K02266]
103231457  PSMB5; proteasome subunit beta 5 [KO:K02737] [EC:3.4.25.1]
103231597  [KO:K02272]
103231882  NDUFB11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
103231971  CACNA1F; calcium voltage-gated channel subunit alpha1 F [KO:K04853]
103232235  COX7B; cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
103233082  CSNK2A2; casein kinase 2 alpha 2 [KO:K03097] [EC:2.7.11.1]
103233205  PSMD7; proteasome 26S subunit, non-ATPase 7 [KO:K03038]
103233293  BAD; BCL2 associated agonist of cell death [KO:K02158]
103233412  [KO:K02263]
103233451  [KO:K08009]
103233496  [KO:K07375]
103233589  GRIN3B; glutamate ionotropic receptor NMDA type subunit 3B [KO:K05214]
103233597  ATP5D; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134]
103233605  NDUFS7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2]
103233614  [KO:K00420]
103233716  CREB3L3; cAMP responsive element binding protein 3 like 3 [KO:K09048]
103233757  NDUFA11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
103233760  [KO:K02128]
103233776  [KO:K07375]
103233777  [KO:K07375]
103233836  NDUFA7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
103234046  NDUFB7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
103234228  NDUFA13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
103234377  [KO:K04345] [EC:2.7.11.11]
103234391  PIK3R2; phosphoinositide-3-kinase regulatory subunit 2 [KO:K02649]
103234417  [KO:K00411] [EC:7.1.1.8]
103234629  PSMD8; proteasome 26S subunit, non-ATPase 8 [KO:K03031]
103234633  RYR1; ryanodine receptor 1 [KO:K04961]
103234690  PSMC4; proteasome 26S subunit, ATPase 4 [KO:K03063]
103234862  KLC3; kinesin light chain 3 [KO:K10407]
103234973  GRIN2D; glutamate ionotropic receptor NMDA type subunit 2D [KO:K05212]
103235004  BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
103235239  NDUFA3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
103235309  [KO:K02267]
103235918  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
103236018  NDUFS3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2]
103236040  PPP3CA; protein phosphatase 3 catalytic subunit alpha [KO:K04348] [EC:3.1.3.16]
103236098  PSMC3; proteasome 26S subunit, ATPase 3 [KO:K03065]
103236222  [KO:K02138]
103236238  SLC25A31; solute carrier family 25 member 31 [KO:K05863]
103236278  NDUFC1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
103236524  [KO:K03938]
103236571  CREB3L1; cAMP responsive element binding protein 3 like 1 [KO:K09048]
103236614  CASP3; caspase 3 [KO:K02187] [EC:3.4.22.56]
103236621  SLC25A4; solute carrier family 25 member 4 [KO:K05863]
103237165  [KO:K00417]
103237200  [KO:K02268]
103237406  NDUFB9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
103237609  [KO:K00413]
103237818  TUBAL3; tubulin alpha like 3 [KO:K07374]
103237855  ATP5C1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136]
103238089  KIF5B; kinesin family member 5B [KO:K10396]
103238260  TUBA1B; tubulin alpha 1b [KO:K07374]
103238261  TUBA1A; tubulin alpha 1a [KO:K07374]
103238262  [KO:K07374]
103238407  ATP5G2; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C2 (subunit 9) [KO:K02128]
103238538  ATP5B; ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide [KO:K02133] [EC:7.1.2.2]
103238561  NDUFA4L2; NDUFA4, mitochondrial complex associated like 2 [KO:K03948]
103238566  DDIT3; DNA damage inducible transcript 3 [KO:K04452]
103238888  NDUFA12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
103238923  APAF1; apoptotic peptidase activating factor 1 [KO:K02084]
103239250  COX6A1; cytochrome c oxidase subunit 6A1 [KO:K02266]
103239294  PSMD9; proteasome 26S subunit, non-ATPase 9 [KO:K06693]
103239501  KIF5A; kinesin family member 5A [KO:K10396]
103239540  [KO:K07375]
103239541  CACNA1B; calcium voltage-gated channel subunit alpha1 B [KO:K04849]
103239564  TUBB4B; tubulin beta 4B class IVb [KO:K07375]
103239575  GRIN1; glutamate ionotropic receptor NMDA type subunit 1 [KO:K05208]
103239621  NOTCH1; notch 1 [KO:K02599]
103239807  PSMA1; proteasome subunit alpha 1 [KO:K02725] [EC:3.4.25.1]
103239827  [KO:K08738]
103239874  HSPA5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
103239889  PSMB7; proteasome subunit beta 7 [KO:K02739] [EC:3.4.25.1]
103240563  FYN; FYN proto-oncogene, Src family tyrosine kinase [KO:K05703] [EC:2.7.10.2]
103240581  [KO:K00237]
103240980  PSMB1; proteasome subunit beta 1 [KO:K02732] [EC:3.4.25.1]
103240990  [KO:K08738]
103241089  COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
103241278  IL1B; interleukin 1 beta [KO:K04519]
103241279  IL1A; interleukin 1 alpha [KO:K04383]
103241350  EIF2AK3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
103241599  PIK3CB; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit beta [KO:K00922] [EC:2.7.1.153]
103241732  PSMD13; proteasome 26S subunit, non-ATPase 13 [KO:K03039]
103241929  STIP1; stress induced phosphoprotein 1 [KO:K09553]
103242203  PSMB6; proteasome subunit beta 6 [KO:K02738] [EC:3.4.25.1]
103242353  [KO:K02267]
103242697  PSMD11; proteasome 26S subunit, non-ATPase 11 [KO:K03036]
103242750  CCL5; C-C motif chemokine ligand 5 [KO:K12499]
103243059  ATP5H; ATP synthase, H+ transporting, mitochondrial Fo complex subunit D [KO:K02138]
103243074  GRIN2C; glutamate ionotropic receptor NMDA type subunit 2C [KO:K05211]
103243081  ADRM1; adhesion regulating molecule 1 [KO:K06691]
103243135  PSMA7; proteasome subunit alpha 7 [KO:K02731] [EC:3.4.25.1]
103243162  PSMD12; proteasome 26S subunit, non-ATPase 12 [KO:K03035]
103243218  PSMC5; proteasome 26S subunit, ATPase 5 [KO:K03066]
103243426  ATP5E; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135]
103243432  TUBB1; tubulin beta 1 class VI [KO:K07375]
103243526  PSMD3; proteasome 26S subunit, non-ATPase 3 [KO:K03033]
103243561  PSMB3; proteasome subunit beta 3 [KO:K02735] [EC:3.4.25.1]
103243629  ATP5G1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C1 (subunit 9) [KO:K02128]
103243885  COX7A2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
103244129  [KO:K03948]
103244190  [KO:K08738]
103244309  [KO:K03947]
103244527  UQCRQ; ubiquinol-cytochrome c reductase complex III subunit VII [KO:K00418]
103244541  VDAC1; voltage dependent anion channel 1 [KO:K05862]
103244608  EGR1; early growth response 1 [KO:K09203]
103244665  NDUFA2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
103245120  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
103245250  PSMA4; proteasome subunit alpha 4 [KO:K02728] [EC:3.4.25.1]
103245320  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
103246111  RYR3; ryanodine receptor 3 [KO:K04963]
103246147  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
103246614  NCF1; neutrophil cytosolic factor 1 [KO:K08011]
103246815  RAC1; ras-related C3 botulinum toxin substrate 1 (rho family, small GTP binding protein Rac1) [KO:K04392]
103247014  PRNP; prion protein [KO:K05634]
103247334  SLC25A5; solute carrier family 25 member 5 [KO:K05863]
103247345  NDUFA1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
103247465  CYBB; cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
103247519  SLC25A6; solute carrier family 25 member 6 [KO:K05863]
103247857  [KO:K07374]
103248003  COX4I2; cytochrome c oxidase subunit 4I2 [KO:K02263]
103248104  NDUFC2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
103248206  [KO:K04565] [EC:1.15.1.1]
103248368  [KO:K04741] [EC:3.4.22.-]
103248461  SDHD; succinate dehydrogenase complex subunit D [KO:K00237]
103248479  NCAM1; neural cell adhesion molecule 1 [KO:K06491]
103248693  HSPA8; heat shock protein family A (Hsp70) member 8 [KO:K03283]
4097488  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
4097489  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
4097490  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
4097491  COX2; cytochrome c oxidase subunit II [KO:K02261]
4097492  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
4097493  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
4097494  COX3; cytochrome c oxidase subunit III [KO:K02262]
4097495  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
4097496  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
4097497  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
4097498  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
4097499  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
4097500  CYTB; cytochrome b [KO:K00412]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
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Saa P, Harris DA, Cervenakova L
  Title
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  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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Neurobiol Dis 142:104973 (2020)
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Reference
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  Title
The highways and byways of prion protein trafficking.
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Trends Cell Biol 15:102-11 (2005)
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Reference
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Nature 443:803-10 (2006)
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J Neurosci Res 75:153-61 (2004)
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  Title
Prion biology relevant to bovine spongiform encephalopathy.
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J Anim Sci 83:1455-76 (2005)
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PMID:27581199
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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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Int J Mol Sci 20:E5107 (2019)
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Int J Mol Sci 20:E6004 (2019)
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Reference
PMID:30710214
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Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
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Mol Neurobiol 56:6035-6045 (2019)
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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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Fine-tuning PERK signaling for neuroprotection.
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J Neurochem 142:812-826 (2017)
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  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
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Pathogens 6:63 (2017)
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Reference
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  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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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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J Cell Mol Med 15:2025-39 (2011)
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The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
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Neurobiol Dis 30:331-42 (2008)
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Reference
PMID:16844381
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Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
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An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
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Neurobiol Dis 23:669-78 (2006)
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Disease-associated prion protein oligomers inhibit the 26S proteasome.
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Reference
PMID:10195903
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Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Reference
PMID:14532116
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Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
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EMBO J 22:5435-45 (2003)
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PMID:31417367
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Zamponi E, Pigino GF
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Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
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Front Cell Neurosci 13:350 (2019)
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Reference
PMID:29261664
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Zamponi E, Buratti F, Cataldi G, Caicedo HH, Song Y, Jungbauer LM, LaDu MJ, Bisbal M, Lorenzo A, Ma J, Helguera PR, Morfini GA, Brady ST, Pigino GF
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Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
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PLoS One 12:e0188340 (2017)
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Reference
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Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
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Understanding the neurospecificity of Prion protein signaling.
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Front Biosci (Landmark Ed) 16:169-86 (2011)
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Reference
PMID:23479001
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Didonna A
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Prion protein and its role in signal transduction.
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Cell Mol Biol Lett 18:209-30 (2013)
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Reference
PMID:28507517
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Shah SZA, Zhao D, Hussain T, Yang L
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The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
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Front Aging Neurosci 9:120 (2017)
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PMID:15528202
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Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
  Title
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
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J Biol Chem 280:1529-34 (2005)
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Reference
PMID:15640354
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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.
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Proc Natl Acad Sci U S A 102:886-91 (2005)
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Reference
PMID:20205843
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Dearmond SJ, Bajsarowicz K
  Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
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Mol Neurodegener 5:6 (2010)
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Reference
PMID:29998397
  Authors
Hirsch TZ, Martin-Lanneree S, Reine F, Hernandez-Rapp J, Herzog L, Dron M, Privat N, Passet B, Halliez S, Villa-Diaz A, Lacroux C, Klein V, Haik S, Andreoletti O, Torres JM, Vilotte JL, Beringue V, Mouillet-Richard S
  Title
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases.
  Journal
Mol Neurobiol 56:2159-2173 (2019)
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Reference
PMID:28714865
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Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
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J Clin Invest 127:3230-3239 (2017)
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)
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Reference
PMID:15611724
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Mallucci G, Collinge J
  Title
Rational targeting for prion therapeutics.
  Journal
Nat Rev Neurosci 6:23-34 (2005)
DOI:10.1038/nrn1584
Related
pathway
csab00190  Oxidative phosphorylation
csab03050  Proteasome
csab04020  Calcium signaling pathway
csab04140  Autophagy - animal
csab04141  Protein processing in endoplasmic reticulum
csab04142  Lysosome
csab04144  Endocytosis
csab04210  Apoptosis
csab04330  Notch signaling pathway
csab04610  Complement and coagulation cascades
csab04668  TNF signaling pathway
KO pathway
ko05020   

DBGET integrated database retrieval system