KEGG   PATHWAY: ssc05020
Entry
ssc05020                    Pathway                                
Name
Prion disease - Sus scrofa (pig)
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
ssc05020  Prion disease
ssc05020

Organism
Sus scrofa (pig) [GN:ssc]
Gene
494014  PRNP; major prion protein precursor [KO:K05634]
100515564  NCAM1; neural cell adhesion molecule 1 isoform X21 [KO:K06491]
100737392  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
100514785  LAMC1; laminin subunit gamma-1 precursor [KO:K05635]
397036  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100623923  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
397652  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
100623628  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
396633  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
791125  FYN; tyrosine-protein kinase Fyn [KO:K05703] [EC:2.7.10.2]
397440  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
100736832  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
100626022  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100515684  GRIN2C; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2C [KO:K05211]
100521797  GRIN2D; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D [KO:K05212]
100153054  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X1 [KO:K05213]
100623799  GRIN3B; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
102164336  CACNA1B; LOW QUALITY PROTEIN: voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
100518733  CACNA1C; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
100511102  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X14 [KO:K04851]
100514745  CACNA1F; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
100622149  CACNA1S; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
407060  HSPA5; 78 kDa glucose-regulated protein isoform X2 [KO:K09490] [EC:3.6.4.10]
100513348  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
100155472  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100144302  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
100240743  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
396718  RYR1; ryanodine receptor 1 [KO:K04961]
396856  RYR2; LOW QUALITY PROTEIN: ryanodine receptor 2 [KO:K04962]
396888  RYR3; LOW QUALITY PROTEIN: ryanodine receptor 3 [KO:K04963]
397454  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
100517279  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
100155015  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
397241  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
100158082  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X4 [KO:K04348] [EC:3.1.3.16]
396603  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X3 [KO:K04348] [EC:3.1.3.16]
100625117  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
100627903  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
100521065  BAD; LOW QUALITY PROTEIN: bcl2-associated agonist of cell death [KO:K02158]
397010  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
397659  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
397651  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
403155  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
100158185  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100517196  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100518564  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100152612  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
100513283  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
100170131  CYCS; cytochrome c [KO:K08738]
100522842  cytochrome c [KO:K08738]
100519295  cytochrome c-like [KO:K08738]
100621347  cytochrome c-like [KO:K08738]
100518913  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
397244  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100153794  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
808501  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
808502  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
808508  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
808510  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
808509  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
808511  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
808512  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100522783  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100037991  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100620809  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
100515069  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100520950  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
100520312  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
100156288  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100579141  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100525371  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
100157442  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
100517634  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100154443  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100524622  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100521478  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
100312976  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100514033  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
100518016  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100622110  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100739619  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100513895  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100525083  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X2 [KO:K03959]
100624335  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100523751  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
733605  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
100737726  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
448979  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
100154130  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100623873  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100522775  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
100516402  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
100156075  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
100510948  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100516161  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
100514655  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100520115  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100624738  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
100521945  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
100037985  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100513030  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
780433  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
414412  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235] [EC:1.3.5.1]
100524676  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
100048954  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
100522678  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
808513  CYTB; cytochrome b [KO:K00412]
100525869  cytochrome c1, heme protein, mitochondrial [KO:K00413]
100156879  cytochrome b-c1 complex subunit 1, mitochondrial isoform X4 [KO:K00414]
100524613  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100524873  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
100157621  UQCRB; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
100524308  cytochrome b-c1 complex subunit 8 [KO:K00418]
100155687  UQCR10; cytochrome b-c1 complex subunit 9 [KO:K00419]
100524239  cytochrome b-c1 complex subunit 10 [KO:K00420]
808507  COX3; cytochrome c oxidase subunit III [KO:K02262]
808503  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
808504  COX2; cytochrome c oxidase subunit II [KO:K02261]
100624067  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
110257420  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
100156967  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
492822  COX5B; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265]
100152232  COX6A1; cytochrome c oxidase subunit VIa polypeptide 1 [KO:K02266]
100626250  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
100038022  COX6B; cytochrome c oxidase subunit 6B1 [KO:K02267]
110261055  cytochrome c oxidase subunit 6B2 [KO:K02267]
100037989  COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
102157773  cytochrome c oxidase subunit 6C-like [KO:K02268]
399685  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial precursor [KO:K02270]
100038000  COX7A2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100516606  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X4 [KO:K02270]
100516480  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100517408  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100037990  COX7C; cytochrome c oxidase subunit 7C, mitochondrial precursor [KO:K02272]
100038031  COX8H; COX8H protein [KO:K02273]
100522725  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
100157935  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
100157880  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100157156  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
102160861  ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100523423  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100134963  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
808506  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100154817  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
574054  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100513629  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100037988  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
100519871  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
100512624  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
733678  ATP5O; ATP synthase subunit O, mitochondrial precursor [KO:K02137]
100622245  ATP5J; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
808505  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100156790  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
100620420  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
397058  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
100513739  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
100626738  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
497063  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100516779  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100154408  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
497065  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100037992  PSMB7; proteasome subunit beta type-7 precursor [KO:K02739] [EC:3.4.25.1]
497064  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100622444  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100155139  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100621969  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100153923  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100514865  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
100155274  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
110260986  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
100154148  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
100512454  PSMC3; 26S protease regulatory subunit 6A isoform X1 [KO:K03065]
397043  PSMC5; 26S protease regulatory subunit 8 [KO:K03066]
100627568  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100153828  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100512253  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100525255  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
100524394  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100523278  PSMD11; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100519870  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100626274  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100517815  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
733583  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
100156327  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
100515483  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
102157770  proteasomal ubiquitin receptor ADRM1 [KO:K06691]
110257483  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
100155621  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
414437  C5; complement C5a anaphylatoxin precursor [KO:K03994]
100037952  C6; complement component C6 precursor [KO:K03995]
397526  C7; complement component C7 precursor [KO:K03996]
100037953  C8A; complement component C8 alpha chain precursor [KO:K03997]
100037954  C8B; complement component C8 beta chain precursor [KO:K03998]
100037955  C8G; complement component C8 gamma chain precursor [KO:K03999]
100037951  C9; complement component C9 precursor [KO:K04000]
445461  C1QA; complement C1q subcomponent subunit A precursor [KO:K03986]
100518846  C1QB; complement C1q subcomponent subunit B precursor [KO:K03987]
445460  C1QC; complement C1q subcomponent subunit C precursor [KO:K03988]
396648  HSP70.2; heat shock 70 kDa protein 1B [KO:K03283]
100144518  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
396906  HSPA6; heat shock 70 kDa protein [KO:K03283]
100511890  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
110258061  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
404693  CAV1; caveolin-1 isoform b [KO:K06278]
100125375  CAV2; caveolin-2 [KO:K12958]
641358  CAV3; caveolin-3 [KO:K12959]
100518131  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
100621789  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
100622559  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
100511937  phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
100739510  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
100623707  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X2 [KO:K02649]
100622955  PRKCD; protein kinase C delta type isoform X2 [KO:K06068] [EC:2.7.11.13]
397108  GP91-PHOX; cytochrome b-245 heavy chain precursor [KO:K21421] [EC:1.-.-.-]
397507  CYBA; cytochrome b-245 light chain [KO:K08009]
100152055  NCF4; neutrophil cytosol factor 4 isoform X3 [KO:K08012]
100134857  NCF1; neutrophil cytosol factor 1 [KO:K08011]
100142665  NCF2; neutrophil cytosol factor 2 [KO:K08010]
100627149  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
100621675  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
574062  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
100156630  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
100155841  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
110260605  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
396610  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100521736  MAPK10; mitogen-activated protein kinase 10 isoform X3 [KO:K04440] [EC:2.7.11.24]
396609  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
100153927  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
445013  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
100736562  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
100513441  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
100142688  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
100142689  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100516663  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
100520468  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
100522979  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100515635  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
100144516  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
100520726  EGR1; early growth response protein 1 [KO:K09203]
396613  CCL5; C-C motif chemokine 5 precursor [KO:K12499]
397094  IL1A; interleukin-1 alpha precursor [KO:K04383]
396565  IL1B2; interleukin 1, beta [KO:K04519]
397122  IL1B; interleukin-1 beta precursor [KO:K04519]
399500  IL6; interleukin-6 precursor [KO:K05405]
397086  TNF; tumor necrosis factor [KO:K03156]
100126852  GSK3B; glycogen synthase kinase-3 beta [KO:K03083] [EC:2.7.11.26]
100517435  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
100737256  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
397211  CSNK2B; casein kinase II subunit beta [KO:K03115]
595132  KIF5B; kinesin-1 heavy chain [KO:K10396]
100622102  KIF5C; kinesin heavy chain [KO:K10396]
100519957  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
100157157  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
100521540  KLC2; kinesin light chain 2 isoform X2 [KO:K10407]
100523621  KLC1; kinesin light chain 1 isoform X7 [KO:K10407]
102158866  KLC3; kinesin light chain 3 [KO:K10407]
733594  TUBA1B; tubulin alpha-1B chain [KO:K07374]
100151951  TUBA4A; tubulin alpha-4A chain [KO:K07374]
100155138  tubulin alpha-3 chain isoform X2 [KO:K07374]
100158003  LOW QUALITY PROTEIN: tubulin alpha-1D chain [KO:K07374]
100127131  tubulin alpha-1C chain isoform X2 [KO:K07374]
100518253  TUBA8; tubulin alpha-8 chain [KO:K07374]
100510930  tubulin alpha-3 chain isoform X2 [KO:K07374]
100135051  TUBA1A; tubulin alpha-1A chain [KO:K07374]
100623355  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
733686  TUBB; tubulin beta chain [KO:K07375]
100134983  TUBB1; tubulin beta-1 chain [KO:K07375]
100153507  TUBB2B; tubulin beta-2B chain [KO:K07375]
100516352  TUBB4B; tubulin beta-4B chain [KO:K07375]
100736584  TUBB4A; tubulin beta-4A chain [KO:K07375]
100624785  tubulin beta-2A chain [KO:K07375]
100623583  TUBB6; LOW QUALITY PROTEIN: 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
  Authors
Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
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Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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Soto C, Satani N
  Title
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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Mays CE, Soto C
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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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Kovacs GG, Budka H
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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
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Prions and their partners in crime.
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Nature 443:803-10 (2006)
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Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
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Reference
PMID:17085779
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Fasano C, Campana V, Zurzolo C
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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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Roucou X, Gains M, LeBlanc AC
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Neuroprotective functions of prion protein.
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J Neurosci Res 75:153-61 (2004)
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Reference
PMID:10398674
  Authors
Harris DA
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Cellular biology of prion diseases.
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Clin Microbiol Rev 12:429-44 (1999)
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.
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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
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Prion Protein in Glioblastoma Multiforme.
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Int J Mol Sci 20:E5107 (2019)
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Reference
PMID:31795242
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
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The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
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Int J Mol Sci 20:E6004 (2019)
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Reference
PMID:30710214
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Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
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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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Reference
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
PMID:28643372
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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
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
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Pathogens 6:E63 (2017)
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Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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Reference
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
PMID:21722302
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Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
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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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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.
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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.
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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.
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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)
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
ssc00190  Oxidative phosphorylation
ssc03050  Proteasome
ssc04020  Calcium signaling pathway
ssc04140  Autophagy - animal
ssc04141  Protein processing in endoplasmic reticulum
ssc04142  Lysosome
ssc04144  Endocytosis
ssc04210  Apoptosis
ssc04330  Notch signaling pathway
ssc04610  Complement and coagulation cascades
ssc04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system