KEGG   PATHWAY: dle05020
Entry
dle05020                    Pathway                                
Name
Prion disease - Delphinapterus leucas (beluga whale)
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
dle05020  Prion disease
dle05020

Organism
Delphinapterus leucas (beluga whale) [GN:dle]
Gene
111165622  PRNP; major prion protein [KO:K05634]
111166865  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
111175214  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
111184636  LAMC1; laminin subunit gamma-1 [KO:K05635]
111172168  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
111183822  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
111178380  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
111166674  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
111180640  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
111164732  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
111176991  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
111163530  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
111176787  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
111182859  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
111180666  GRIN2D; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D [KO:K05212]
111188066  GRIN3A; glutamate receptor ionotropic, NMDA 3A [KO:K05213]
111170472  GRIN3B; glutamate receptor ionotropic, NMDA 3B isoform X1 [KO:K05214]
111176963  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
111176594  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
111174277  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
111169490  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
111168340  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
111177217  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
111187362  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
111173047  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
111186212  ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374]
111173840  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
111180892  RYR1; LOW QUALITY PROTEIN: ryanodine receptor 1 [KO:K04961]
111163908  RYR2; ryanodine receptor 2 isoform X1 [KO:K04962]
111181786  RYR3; ryanodine receptor 3 isoform X1 [KO:K04963]
111174169  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
111176861  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
111178692  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
111179107  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111183521  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111169035  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111186607  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
111188120  PPP3R2; LOW QUALITY PROTEIN: calcineurin subunit B type 2 [KO:K06268]
111183810  BAD; bcl2-associated agonist of cell death [KO:K02158]
111182441  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
111183542  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
111173555  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
111187864  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 3-like [KO:K15041]
111168263  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
111173410  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
111169059  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
111165889  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
111183554  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
111187821  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
111180543  cytochrome c [KO:K08738]
111174575  cytochrome c-like [KO:K08738]
111174031  cytochrome c-like [KO:K08738]
111172911  cytochrome c 2 [KO:K08738]
111174197  cytochrome c-like [KO:K08738]
111175930  cytochrome c-like [KO:K08738]
111187099  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
111173401  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
111183499  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
31412977  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
31412980  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
31412989  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
31413004  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
31412990  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
31412993  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
31413006  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
111183658  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
111172449  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial-like [KO:K03943] [EC:7.1.1.2]
111162874  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
111163814  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
111182541  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111181038  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111181538  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111173848  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
111178932  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
111186230  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
111166115  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
111177253  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
111176645  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
111171993  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
111182239  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
111166137  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
111168714  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
111166656  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
111175981  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111164113  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111177666  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
111181221  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
111171723  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
111168836  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
111175937  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
111173776  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
111163197  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
111166580  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
111172389  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
111164367  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
111185164  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
111165552  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
111171760  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
111166990  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
111183956  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
111186772  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
111175620  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
111178547  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
111170487  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
111183653  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
111164871  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
111184910  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
111178524  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
111187104  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
111166971  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
111166859  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
111173600  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
111180964  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
31412994  CYTB; cytochrome b [KO:K00412]
111165123  cytochrome c1, heme protein, mitochondrial [KO:K00413]
111174428  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
111182267  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
111175766  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
111180406  cytochrome b-c1 complex subunit 7 [KO:K00417]
111182430  cytochrome b-c1 complex subunit 8 [KO:K00418]
111162798  cytochrome b-c1 complex subunit 9 [KO:K00419]
111166133  cytochrome b-c1 complex subunit 10 [KO:K00420]
31413001  COX3; cytochrome c oxidase subunit III [KO:K02262]
31412985  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
31412987  COX2; cytochrome c oxidase subunit II [KO:K02261]
111179428  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
111184329  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
111177826  cytochrome c oxidase subunit 5A, mitochondrial isoform X1 [KO:K02264]
111187511  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
111182105  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
111162636  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
111181225  cytochrome c oxidase subunit 6B1 [KO:K02267]
111180991  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6B2 [KO:K02267]
111180389  cytochrome c oxidase subunit 6C [KO:K02268]
111177953  cytochrome c oxidase subunit 6C [KO:K02268]
111173965  cytochrome c oxidase subunit 6C-like [KO:K02268]
111183230  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
111181212  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
111174833  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
111178277  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111171524  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
111162930  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111172029  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
111170285  cytochrome c oxidase subunit 8B, mitochondrial-like [KO:K02273]
111183826  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
111168206  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
111173862  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
111177988  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
111170529  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
111178333  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
111186725  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
31413000  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
111180038  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
111172892  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
111166314  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
111173931  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
111182847  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
111163853  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
111174841  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
31412988  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
111173226  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
111184500  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
111177869  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
111181484  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
111186806  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
111180078  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
111165265  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
111173114  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
111185965  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
111177231  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
111166412  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
111175568  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
111164181  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
111168047  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
111169730  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
111164612  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
111176166  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
111180860  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
111173152  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
111183961  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
111182616  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
111173238  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
111171923  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
111166341  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
111186839  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
111182644  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
111185761  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
111174220  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
111179575  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
111170282  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
111169718  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
111172793  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
111180900  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
111186817  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
111181329  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
111177265  C5; complement C5 isoform X1 [KO:K03994]
111187127  C6; complement component C6 isoform X1 [KO:K03995]
111187150  C7; complement component C7 [KO:K03996]
111175863  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
111175865  C8B; complement component C8 beta chain isoform X1 [KO:K03998]
111177008  C8G; complement component C8 gamma chain isoform X2 [KO:K03999]
111187275  C9; complement component C9 [KO:K04000]
111163328  C1QA; complement C1q subcomponent subunit A [KO:K03986]
111163327  C1QB; complement C1q subcomponent subunit B [KO:K03987]
111163329  C1QC; complement C1q subcomponent subunit C [KO:K03988]
111170662  heat shock 70 kDa protein 1B [KO:K03283]
111170663  heat shock 70 kDa protein 1B [KO:K03283]
111170664  heat shock 70 kDa protein 1-like [KO:K03283]
111167407  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
111170417  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
111177023  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
111179315  CAV1; caveolin-1 isoform X1 [KO:K06278]
111179321  CAV2; caveolin-2 [KO:K12958]
111169961  CAV3; caveolin-3 [KO:K12959]
111173839  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
111179235  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
111187262  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922] [EC:2.7.1.153]
111166652  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
111185984  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
111175760  phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
111174287  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
111170450  cytochrome b-245 heavy chain isoform X1 [KO:K21421] [EC:1.-.-.-]
111179403  cytochrome b-245 light chain isoform X1 [KO:K08009]
111186386  neutrophil cytosol factor 4-like [KO:K08012]
111187847  neutrophil cytosol factor 4 isoform X1 [KO:K08012]
111182060  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
111184640  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
111181886  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
111186216  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
111178736  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
111178738  MAPK13; mitogen-activated protein kinase 13 isoform X2 [KO:K04441] [EC:2.7.11.24]
111186361  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
111186364  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
111168108  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
111169265  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
111169895  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
111163022  MAPK1; mitogen-activated protein kinase 1 isoform X1 [KO:K04371] [EC:2.7.11.24]
111182181  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
111171769  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
111172890  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
111167181  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
111188007  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
111166195  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
111177962  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
111183987  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
111180225  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
111170946  ATF6B; LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
111182498  EGR1; early growth response protein 1 [KO:K09203]
111163675  CCL5; C-C motif chemokine 5 [KO:K12499]
111187501  IL1A; interleukin-1 alpha [KO:K04383]
111187492  IL1B; interleukin-1 beta [KO:K04519]
111181978  IL6; interleukin-6 [KO:K05405]
111170657  TNF; tumor necrosis factor [KO:K03156]
111168040  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
111184343  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
111165308  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
111170713  casein kinase II subunit beta isoform X1 [KO:K03115]
111172744  KIF5C; kinesin heavy chain [KO:K10396]
111173834  KIF5A; kinesin heavy chain isoform X2 [KO:K10396]
111178120  KIF5B; kinesin-1 heavy chain [KO:K10396]
111176031  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
111178841  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
111183714  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
111180743  KLC3; kinesin light chain 3 [KO:K10407]
111163074  tubulin alpha-3 chain-like [KO:K07374]
111162959  tubulin alpha-3 chain [KO:K07374]
111171848  tubulin alpha-4A chain [KO:K07374]
111171851  tubulin alpha-1 chain-like [KO:K07374]
111170121  tubulin alpha chain-like 3 [KO:K07374]
111176582  TUBA8; tubulin alpha-8 chain isoform X2 [KO:K07374]
111174050  tubulin alpha-1A chain [KO:K07374]
111174051  tubulin alpha-1B chain [KO:K07374]
111174052  tubulin alpha-1C chain-like [KO:K07374]
111176583  tubulin alpha-8 chain [KO:K07374]
111176980  TUBB4B; tubulin beta-4B chain [KO:K07375]
111170669  TUBB; tubulin beta chain [KO:K07375]
111166070  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
111181308  TUBB2A; tubulin beta-2A chain [KO:K07375]
111181312  tubulin beta-2B chain [KO:K07375]
111186724  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
111179686  tubulin beta-3 chain [KO:K07375]
111163019  TUBB6; tubulin beta-6 chain [KO:K07375]
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
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
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Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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Soto C, Satani N
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The intricate mechanisms of neurodegeneration in prion diseases.
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Trends Mol Med 17:14-24 (2011)
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Reference
PMID:27060771
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The stress of prion disease.
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Brain Res 1648:553-560 (2016)
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Reference
PMID:32565065
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Jones E, Mead S
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Genetic risk factors for Creutzfeldt-Jakob disease.
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Neurobiol Dis 142:104973 (2020)
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Reference
PMID:18245809
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Prion diseases: from protein to cell pathology.
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Am J Pathol 172:555-65 (2008)
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Reference
PMID:15695097
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
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Trends Cell Biol 15:102-11 (2005)
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Reference
PMID:17051207
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Caughey B, Baron GS
  Title
Prions and their partners in crime.
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Nature 443:803-10 (2006)
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Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
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Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
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Reference
PMID:17085779
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Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
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J Mol Neurosci 29:195-214 (2006)
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Reference
PMID:14705136
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J Neurosci Res 75:153-61 (2004)
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Reference
PMID:10398674
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Cellular biology of prion diseases.
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Reference
PMID:15890824
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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)
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Reference
PMID:27581199
  Authors
Peggion C, Bertoli A, Sorgato MC
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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
PMID:31618844
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Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
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Int J Mol Sci 20:E5107 (2019)
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Reference
PMID:31795242
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Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
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The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
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Int J Mol Sci 20:E6004 (2019)
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Reference
PMID:30710214
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Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
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Mol Neurobiol 56:6035-6045 (2019)
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PMID:23439666
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Song Z, Zhao D, Yang L
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Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
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Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
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Reference
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Halliday M, Hughes D, Mallucci GR
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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
PMID:29194372
  Authors
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  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
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Pathogens 6:E63 (2017)
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Reference
PMID:24619348
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Hetz C, Mollereau B
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Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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Reference
PMID:29476642
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
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FEBS J 286:342-355 (2019)
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Reference
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Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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J Cell Mol Med 15:2025-39 (2011)
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Reference
PMID:18420416
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Ferreiro E, Oliveira CR, Pereira CM
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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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Reference
PMID:21980554
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Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
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Commun Integr Biol 4:258-61 (2011)
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Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
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Exp Mol Med 47:e147 (2015)
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Reference
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Disease-associated prion protein oligomers inhibit the 26S proteasome.
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Mol Cell 26:175-88 (2007)
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Reference
PMID:10195903
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Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
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Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Science 284:339-43 (1999)
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Reference
PMID:14532116
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Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
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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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Reference
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
  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)
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Reference
PMID:21196165
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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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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)
DOI:10.1007/s12035-018-1193-7
Reference
PMID:28714865
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
PMID:15342004
  Authors
Mabbott NA
  Title
The complement system in prion diseases.
  Journal
Curr Opin Immunol 16:587-93 (2004)
DOI:10.1016/j.coi.2004.07.002
Reference
PMID:15611724
  Authors
Mallucci G, Collinge J
  Title
Rational targeting for prion therapeutics.
  Journal
Nat Rev Neurosci 6:23-34 (2005)
DOI:10.1038/nrn1584
Related
pathway
dle00190  Oxidative phosphorylation
dle03050  Proteasome
dle04020  Calcium signaling pathway
dle04140  Autophagy - animal
dle04141  Protein processing in endoplasmic reticulum
dle04142  Lysosome
dle04144  Endocytosis
dle04210  Apoptosis
dle04330  Notch signaling pathway
dle04610  Complement and coagulation cascades
dle04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system