KEGG   PATHWAY: cfa05020
Entry
cfa05020                    Pathway                                
Name
Prion disease - Canis lupus familiaris (dog)
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
cfa05020  Prion disease
cfa05020

Disease
H00061  Prion disease
Organism
Canis lupus familiaris (dog) [GN:cfa]
Gene
100192385  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
100682717  [KO:K02128]
100682783  [KO:K02131]
100682956  [KO:K03948]
100683076  [KO:K02271]
100683530  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100683681  [KO:K02725] [EC:3.4.25.1]
100683682  [KO:K03955]
100683828  [KO:K00417]
100683846  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100683951  [KO:K03949]
100684768  [KO:K03948]
100684842  [KO:K02271]
100684983  [KO:K02271]
100684996  [KO:K02138]
100685007  [KO:K02128]
100685227  [KO:K02128]
100685720  [KO:K02131]
100686423  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100686432  [KO:K02725] [EC:3.4.25.1]
100686500  [KO:K02270]
100686510  [KO:K02271]
100686569  [KO:K02730] [EC:3.4.25.1]
100686580  [KO:K02267]
100686627  RYR3; ryanodine receptor 3 isoform X1 [KO:K04963]
100686889  [KO:K03948]
100687033  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
100687060  [KO:K02730] [EC:3.4.25.1]
100687152  [KO:K03065]
100687259  [KO:K03064]
100687434  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100687556  [KO:K02728] [EC:3.4.25.1]
100688636  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100688724  [KO:K03948]
100688835  [KO:K03949]
100689004  [KO:K08738]
100855591  KLC1; kinesin light chain 1 isoform X10 [KO:K10407]
100855613  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100855651  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396] [EC:5.6.1.3]
100855659  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100855712  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
100855815  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
100855877  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
100855914  [KO:K03947]
100855975  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X2 [KO:K03959]
100855986  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
100855995  [KO:K07374]
100856334  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100856339  [KO:K02649]
100856348  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100856395  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
100856405  TUBA1C; tubulin alpha-1C chain [KO:K07374]
102151342  [KO:K02138]
102151506  [KO:K03947]
102151754  [KO:K02272]
102152785  [KO:K02271]
102152873  [KO:K05862]
102152879  [KO:K02725] [EC:3.4.25.1]
102153059  [KO:K02272]
102153202  TUBB4B; tubulin beta-4B chain isoform X2 [KO:K07375]
102153397  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
102154025  [KO:K03945]
102154035  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
102154194  TUBB3; tubulin beta-3 chain [KO:K07375]
102154372  [KO:K02128]
102154590  [KO:K03961]
102155410  [KO:K02272]
102156642  [KO:K02267]
102156968  [KO:K02266]
106557476  [KO:K07374]
106557778  [KO:K03945]
106558390  [KO:K02273]
111096466  [KO:K03957]
119863903  [KO:K02270]
119866377  [KO:K07374]
119870103  [KO:K03949]
119870484  [KO:K02138]
119870901  [KO:K02267]
119873859  [KO:K03283]
119874008  [KO:K03948]
119874448  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
403486  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
403516  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X17 [KO:K04850]
403522  CCL5; C-C motif chemokine 5 precursor [KO:K12499]
403523  BAX; apoptosis regulator BAX [KO:K02159]
403556  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
403559  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
403567  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
403612  HSP70; heat shock 70 kDa protein 1 [KO:K03283]
403615  RYR2; ryanodine receptor 2 isoform X1 [KO:K04962]
403669  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
403782  IL1A; interleukin-1 alpha [KO:K04383]
403856  MAPK14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
403922  TNF; tumor necrosis factor [KO:K03156]
403955  RAC1; ras-related C3 botulinum toxin substrate 1 precursor [KO:K04392]
403974  IL1B; interleukin-1 beta [KO:K04519]
403980  CAV1; caveolin-1 [KO:K06278]
403985  IL6; interleukin-6 precursor [KO:K05405]
404305  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
474416  KIF5A; kinesin heavy chain isoform X1 [KO:K10396] [EC:5.6.1.3]
474483  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
474503  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
474567  COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
474652  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
474681  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
474789  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X5 [KO:K05213]
474816  C5; complement C5 isoform X1 [KO:K03994]
474830  TUBB; tubulin beta chain [KO:K07375]
474837  STIP1; stress-induced-phosphoprotein 1 isoform X2 [KO:K09553]
474844  CSNK2B; casein kinase II subunit beta isoform X2 [KO:K03115]
474850  [KO:K03283]
474855  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
474909  KLC4; kinesin light chain 4 [KO:K10407]
475032  FYN; tyrosine-protein kinase Fyn isoform X2 [KO:K05703] [EC:2.7.10.2]
475040  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
475094  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
475121  CYC1; cytochrome c1, heme protein, mitochondrial [KO:K00413]
475214  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
475258  CYCS; cytochrome c [KO:K08738]
475266  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X5 [KO:K09047]
475294  CAV2; caveolin-2 [KO:K12958]
475322  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
475338  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
475428  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
475630  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
475739  COX7A2L; cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X3 [KO:K02270]
475756  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X2 [KO:K08860] [EC:2.7.11.1]
475848  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
475870  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
475896  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
475934  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
475978  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
475980  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
476004  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
476040  COX8A; cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
476144  KIF5C; kinesin heavy chain [KO:K10396] [EC:5.6.1.3]
476470  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
476503  UQCRFS1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
476548  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
476659  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
476686  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
476722  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
476735  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
476754  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
476756  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
476794  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
476867  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
477085  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
477185  CSNK2A1; casein kinase II subunit alpha isoform X1 [KO:K03097] [EC:2.7.11.1]
477467  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
477508  [KO:K02266]
477570  [KO:K07374]
477575  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
477595  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
477718  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
477746  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
477798  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
478009  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
478032  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
478108  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
478194  C1QA; complement C1q subcomponent subunit A [KO:K03986]
478217  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
478370  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
478410  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
478421  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
478464  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
478522  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
478575  GSK3B; glycogen synthase kinase-3 beta isoform X13 [KO:K03083] [EC:2.7.11.26]
478577  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
478629  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
478634  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
478639  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
478654  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
478701  TUBB2A; tubulin beta-2A chain [KO:K07375]
478702  [KO:K07375]
478765  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
478806  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
478807  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
478880  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
478918  TUBA4A; tubulin alpha-4A chain isoform X2 [KO:K07374]
478981  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
478983  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
479248  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
479255  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
479335  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X11 [KO:K03937]
479348  C6; complement component C6 [KO:K03995]
479354  C9; complement component C9 [KO:K04000]
479406  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
479435  NCAM1; neural cell adhesion molecule 1 isoform 3 precursor [KO:K06491]
479458  CASP12; caspase-12 [KO:K04741] [EC:3.4.22.-]
479555  C8B; complement component C8 beta chain [KO:K03998]
479623  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
479671  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
479780  COX6A2; cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
479805  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
479815  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
479887  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
479975  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X2 [KO:K04345] [EC:2.7.11.11]
480034  LAMC1; laminin subunit gamma-1 [KO:K05635]
480036  NCF2; neutrophil cytosol factor 2 [KO:K08010]
480149  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
480204  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2 [KO:K03943] [EC:7.1.1.2]
480213  TUBB6; tubulin beta-6 chain [KO:K07375]
480290  PSMA6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
480338  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
480361  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
480465  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X2 [KO:K03035]
480478  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
480537  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
480610  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
480660  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
480672  C8G; complement component C8 gamma chain isoform X2 [KO:K03999]
480676  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
480726  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
480741  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
480830  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
480894  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
480915  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
481033  [KO:K03945]
481271  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
481277  NCF4; neutrophil cytosol factor 4 isoform X2 [KO:K08012]
481528  EGR1; early growth response protein 1 [KO:K09203]
481746  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
482600  [KO:K02138]
482620  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
483302  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
483636  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
483715  KLC2; kinesin light chain 2 [KO:K10407]
483763  BAD; bcl2-associated agonist of cell death [KO:K02158]
483832  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
484405  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
484448  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
484671  CAV3; caveolin-3 [KO:K12959]
484700  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X2 [KO:K03037]
484724  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X15 [KO:K04851]
485046  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
485092  GRIN3B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 3B isoform X1 [KO:K05214]
485783  PRNP; major prion protein precursor [KO:K05634]
485825  COX4I2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
485950  TUBB1; tubulin beta-1 chain [KO:K07375]
486347  UQCR10; cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
486628  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X2 [KO:K04959]
486762  TUBA8; tubulin alpha-8 chain isoform X12 [KO:K07374]
487148  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
487235  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
487381  C1QB; complement C1q subcomponent subunit B [KO:K03987]
487382  C1QC; complement C1q subcomponent subunit C [KO:K03988]
487432  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
487689  [KO:K02132]
487705  NCAM2; neural cell adhesion molecule 2 isoform X2 [KO:K06491]
488084  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
489042  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
489221  C7; complement component C7 isoform X2 [KO:K03996]
489644  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X3 [KO:K00922] [EC:2.7.1.153]
489664  CYBA; cytochrome b-245 alpha chain [KO:K08009]
490012  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
490244  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X2 [KO:K04857]
491018  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
491061  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
491231  [KO:K07375]
491369  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
491825  CYBB; cytochrome b-245, beta polypeptide [KO:K21421] [EC:1.-.-.-]
492093  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
494005  PRKCD; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
494007  GRIN1; glutamate receptor ionotropic, NMDA 1 precursor [KO:K05208]
494009  GRIN2B; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210]
606491  RYR1; ryanodine receptor 1 [KO:K04961]
606963  VDAC3; voltage-dependent anion-selective channel protein 3 isoform 1 [KO:K15041]
607019  MAPK11; mitogen-activated protein kinase 11 isoform X2 [KO:K04441] [EC:2.7.11.24]
607023  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
607182  [KO:K03283]
607407  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X2 [KO:K03948]
607439  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
607466  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
607700  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
607922  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
607943  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
608051  [KO:K07374]
608244  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10 isoform X1 [KO:K03954]
608455  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
608530  UQCRH; cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
608719  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
609027  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X3 [KO:K04348] [EC:3.1.3.16]
609744  UQCRQ; cytochrome b-c1 complex subunit 8 [KO:K00418]
609956  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
609990  [KO:K02272]
610188  [KO:K02725] [EC:3.4.25.1]
610413  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
610453  PPP3R2; calcineurin subunit B type 2 isoform X5 [KO:K06268]
610502  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
610636  [KO:K07374]
610725  [KO:K02128]
611791  UQCR11; cytochrome b-c1 complex subunit 10 [KO:K00420]
611934  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X2 [KO:K09048]
612303  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
612559  PPP3R1; calcineurin subunit B type 1 isoform X2 [KO:K06268]
612614  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
612644  [KO:K02267]
612821  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
804476  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
804477  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
804478  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
804479  COX2; cytochrome c oxidase subunit II [KO:K02261]
804480  COX3; cytochrome c oxidase subunit III [KO:K02262]
804481  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
804482  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
804483  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
804484  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
804485  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
804486  CYTB; cytochrome b [KO:K00412]
804487  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
804488  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
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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Expert Rev Mol Med 18:e5 (2016)
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Reference
PMID:25584786
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Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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The intricate mechanisms of neurodegeneration in prion diseases.
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Trends Mol Med 17:14-24 (2011)
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Reference
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The stress of prion disease.
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Reference
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  Title
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Reference
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Prion biology relevant to bovine spongiform encephalopathy.
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J Anim Sci 83:1455-76 (2005)
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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
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Int J Mol Sci 20:E6004 (2019)
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Reference
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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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Reference
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  Title
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Reference
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Nat Rev Neurosci 15:233-49 (2014)
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Reference
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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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Reference
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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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Reference
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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
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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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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
  Title
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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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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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.
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J Biol Chem 280:1529-34 (2005)
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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.
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Proc Natl Acad Sci U S A 102:886-91 (2005)
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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)
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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
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
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Reference
PMID:15342004
  Authors
Mabbott NA
  Title
The complement system in prion diseases.
  Journal
Curr Opin Immunol 16:587-93 (2004)
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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
cfa00190  Oxidative phosphorylation
cfa03050  Proteasome
cfa04020  Calcium signaling pathway
cfa04140  Autophagy - animal
cfa04141  Protein processing in endoplasmic reticulum
cfa04142  Lysosome
cfa04144  Endocytosis
cfa04210  Apoptosis
cfa04330  Notch signaling pathway
cfa04610  Complement and coagulation cascades
cfa04668  TNF signaling pathway
KO pathway
ko05020   

DBGET integrated database retrieval system