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

Organism
Canis lupus familiaris (dog) [GN:cfa]
Gene
485783  PRNP; major prion protein precursor [KO:K05634]
479435  NCAM1; neural cell adhesion molecule 1 isoform 3 precursor [KO:K06491]
487705  NCAM2; neural cell adhesion molecule 2 isoform X2 [KO:K06491]
480034  LAMC1; laminin subunit gamma-1 [KO:K05635]
403559  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
474837  STIP1; stress-induced-phosphoprotein 1 isoform X2 [KO:K09553]
403556  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
479975  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X2 [KO:K04345] [EC:2.7.11.11]
403523  BAX; apoptosis regulator BAX [KO:K02159]
475032  FYN; tyrosine-protein kinase Fyn isoform X2 [KO:K05703] [EC:2.7.10.2]
494007  GRIN1; glutamate receptor ionotropic, NMDA 1 precursor [KO:K05208]
490012  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
494009  GRIN2B; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210]
483302  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
484405  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
474789  GRIN3A; glutamate receptor ionotropic, NMDA 3A isoform X5 [KO:K05213]
485092  GRIN3B; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 3B isoform X1 [KO:K05214]
480660  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
403516  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X17 [KO:K04850]
484724  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X15 [KO:K04851]
480915  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
490244  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X2 [KO:K04857]
480726  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
475756  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X2 [KO:K08860] [EC:2.7.11.1]
480361  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
474503  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
607439  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
606491  RYR1; ryanodine receptor 1 [KO:K04961]
403615  RYR2; ryanodine receptor 2 isoform X1 [KO:K04962]
100686627  RYR3; ryanodine receptor 3 isoform X1 [KO:K04963]
476548  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
486628  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X2 [KO:K04959]
481746  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
479458  CASP12; caspase-12 [KO:K04741] [EC:3.4.22.-]
609027  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X3 [KO:K04348] [EC:3.1.3.16]
403486  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
479248  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
612559  PPP3R1; calcineurin subunit B type 1 isoform X2 [KO:K06268]
610453  PPP3R2; calcineurin subunit B type 2 isoform X5 [KO:K06268]
483763  BAD; bcl2-associated agonist of cell death [KO:K02158]
474681  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
102152873  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
479255  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
606963  VDAC3; voltage-dependent anion-selective channel protein 3 isoform 1 [KO:K15041]
475630  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
480830  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
492093  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
483832  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
610502  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
482620  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
475258  CYCS; cytochrome c [KO:K08738]
100689004  LOW QUALITY PROTEIN: cytochrome c-like [KO:K08738]
487432  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
403567  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
489042  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
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]
804481  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
804483  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
804482  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [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]
476004  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
480204  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2 [KO:K03943] [EC:7.1.1.2]
478421  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
102154035  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
481033  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
102154025  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
106557778  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
478032  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
100855914  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
102151506  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
607407  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X2 [KO:K03948]
100856334  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100682956  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100684768  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100686889  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100688724  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
119874008  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
475214  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100683951  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
100688835  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
119870103  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
474483  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
476722  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
480741  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
477718  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
608244  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10 isoform X1 [KO:K03954]
479805  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100683682  acyl carrier protein, mitochondrial-like [KO:K03955]
476735  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
475428  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
476659  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
111096466  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100683846  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100855975  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X2 [KO:K03959]
478577  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
478639  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
102154590  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial-like [KO:K03961]
100687033  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
476686  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
477798  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
475094  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
479887  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
480894  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
478880  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
478981  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
475978  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
479335  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X11 [KO:K03937]
475322  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
478629  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
476754  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
610413  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
607943  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
476794  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
478634  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
478217  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
478983  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
476503  UQCRFS1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
804486  CYTB; cytochrome b [KO:K00412]
475121  CYC1; cytochrome c1, heme protein, mitochondrial [KO:K00413]
608455  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
479815  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
608530  UQCRH; cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
100683828  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 7-like [KO:K00417]
609744  UQCRQ; cytochrome b-c1 complex subunit 8 [KO:K00418]
486347  UQCR10; cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
611791  UQCR11; cytochrome b-c1 complex subunit 10 [KO:K00420]
804480  COX3; cytochrome c oxidase subunit III [KO:K02262]
804478  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
804479  COX2; cytochrome c oxidase subunit II [KO:K02261]
479623  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
485825  COX4I2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
478370  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
474567  COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
479780  COX6A2; cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
477508  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
102156968  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
612644  cytochrome c oxidase subunit 6B1 [KO:K02267]
100686580  cytochrome c oxidase subunit 6B1-like [KO:K02267]
102156642  cytochrome c oxidase subunit 6B1-like [KO:K02267]
119870901  cytochrome c oxidase subunit 6B2 [KO:K02267]
475739  COX7A2L; cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X3 [KO:K02270]
612614  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
119863903  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
100686500  cytochrome c oxidase subunit 7A2, mitochondrial isoform X4 [KO:K02270]
100684983  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100687434  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100684842  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
102152785  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100686510  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100683076  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
609990  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
102155410  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
102151754  cytochrome c oxidase subunit 7C, mitochondrial-like isoform X5 [KO:K02272]
102153059  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
476040  COX8A; cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
106558390  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
480149  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
487689  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
403669  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
478009  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
476756  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
102153397  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
804488  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
491061  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
477595  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
478807  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100682717  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100685007  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
100685227  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
102154372  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
610725  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
102151342  ATP synthase subunit d, mitochondrial-like [KO:K02138]
482600  ATP synthase subunit d, mitochondrial-like isoform X2 [KO:K02138]
100684996  ATP synthase subunit d, mitochondrial [KO:K02138]
119870484  ATP synthase subunit d, mitochondrial [KO:K02138]
478410  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100682783  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
100685720  LOW QUALITY PROTEIN: ATP synthase-coupling factor 6, mitochondrial-like isoform X2 [KO:K02131]
804487  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
480290  PSMA6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
100686569  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
100687060  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
475870  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100687556  LOW QUALITY PROTEIN: proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
119874448  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
404305  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100856395  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
100856348  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
476867  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
102152879  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
100683681  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
100686432  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
610188  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
480338  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
607466  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100686423  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
480537  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
475338  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
100855659  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
475040  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
475848  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
475896  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100688636  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100855613  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
478522  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100687259  26S proteasome regulatory subunit 10B [KO:K03064]
475980  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
100687152  26S proteasome regulatory subunit 6A-like [KO:K03065]
480478  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
478654  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100683530  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
491018  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
477467  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
480465  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X2 [KO:K03035]
480610  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
484700  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X2 [KO:K03037]
479671  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
475934  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100192385  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
478765  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
476470  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100855986  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
608719  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
474816  C5; complement C5 isoform X1 [KO:K03994]
479348  C6; complement component C6 [KO:K03995]
489221  C7; complement component C7 isoform X2 [KO:K03996]
100855712  C8A; complement component C8 alpha chain isoform X1 [KO:K03997]
479555  C8B; complement component C8 beta chain [KO:K03998]
480672  C8G; complement component C8 gamma chain isoform X2 [KO:K03999]
479354  C9; complement component C9 [KO:K04000]
478194  C1QA; complement C1q subcomponent subunit A [KO:K03986]
487381  C1QB; complement C1q subcomponent subunit B [KO:K03987]
487382  C1QC; complement C1q subcomponent subunit C [KO:K03988]
474850  heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
403612  HSP70; heat shock 70 kDa protein 1 [KO:K03283]
479406  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
119873859  LOW QUALITY PROTEIN: heat shock cognate 71 kDa protein-like isoform X2 [KO:K03283]
607182  heat shock cognate 71 kDa protein-like [KO:K03283]
480676  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
403980  CAV1; caveolin-1 [KO:K06278]
475294  CAV2; caveolin-2 [KO:K12958]
484671  CAV3; caveolin-3 [KO:K12959]
488084  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
489644  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X3 [KO:K00922] [EC:2.7.1.153]
477085  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
609956  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
487235  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
100856339  phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
494005  PRKCD; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
491825  CYBB; cytochrome b-245, beta polypeptide [KO:K21421] [EC:1.-.-.-]
489664  CYBA; cytochrome b-245 alpha chain [KO:K08009]
481277  NCF4; neutrophil cytosol factor 4 isoform X2 [KO:K08012]
607700  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
480036  NCF2; neutrophil cytosol factor 2 [KO:K08010]
403955  RAC1; ras-related C3 botulinum toxin substrate 1 precursor [KO:K04392]
481271  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
403856  MAPK14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
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]
612821  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
477746  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
478464  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
474652  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
477575  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
100855877  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
607922  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
478806  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
611934  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X2 [KO:K09048]
483636  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
491369  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
485046  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
612303  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
475266  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X5 [KO:K09047]
474855  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
481528  EGR1; early growth response protein 1 [KO:K09203]
403522  CCL5; C-C motif chemokine 5 precursor [KO:K12499]
403782  IL1A; interleukin-1 alpha [KO:K04383]
403974  IL1B; interleukin-1 beta [KO:K04519]
403985  IL6; interleukin-6 precursor [KO:K05405]
403922  TNF; tumor necrosis factor [KO:K03156]
478575  GSK3B; glycogen synthase kinase-3 beta isoform X13 [KO:K03083] [EC:2.7.11.26]
477185  CSNK2A1; casein kinase II subunit alpha isoform X1 [KO:K03097] [EC:2.7.11.1]
478108  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
474844  CSNK2B; casein kinase II subunit beta isoform X2 [KO:K03115]
474416  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
476144  KIF5C; kinesin heavy chain [KO:K10396]
100855651  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
474909  KLC4; kinesin light chain 4 [KO:K10407]
483715  KLC2; kinesin light chain 2 [KO:K10407]
484448  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
100855591  KLC1; kinesin light chain 1 isoform X10 [KO:K10407]
478918  TUBA4A; tubulin alpha-4A chain isoform X2 [KO:K07374]
486762  TUBA8; tubulin alpha-8 chain isoform X12 [KO:K07374]
608051  tubulin alpha-3 chain [KO:K07374]
477570  tubulin alpha-3 chain [KO:K07374]
487148  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
610636  tubulin alpha-1B chain isoform X2 [KO:K07374]
100856405  TUBA1C; tubulin alpha-1C chain [KO:K07374]
106557476  tubulin alpha-1A chain [KO:K07374]
100855995  tubulin alpha-8 chain-like isoform X3 [KO:K07374]
119866377  tubulin alpha-1B chain [KO:K07374]
474830  TUBB; tubulin beta chain [KO:K07375]
485950  TUBB1; tubulin beta-1 chain [KO:K07375]
480213  TUBB6; tubulin beta-6 chain [KO:K07375]
478702  tubulin beta-2B chain [KO:K07375]
491231  LOW QUALITY PROTEIN: tubulin beta chain [KO:K07375]
478701  TUBB2A; tubulin beta-2A chain [KO:K07375]
100855815  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
102153202  TUBB4B; tubulin beta-4B chain isoform X2 [KO:K07375]
102154194  TUBB3; tubulin beta-3 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)
DOI:10.1017/erm.2016.8
Reference
PMID:25584786
  Authors
Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
  Journal
Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
Reference
PMID:20889378
  Authors
Soto C, Satani N
  Title
The intricate mechanisms of neurodegeneration in prion diseases.
  Journal
Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
PMID:27060771
  Authors
Mays CE, Soto C
  Title
The stress of prion disease.
  Journal
Brain Res 1648:553-560 (2016)
DOI:10.1016/j.brainres.2016.04.009
Reference
PMID:32565065
  Authors
Jones E, Mead S
  Title
Genetic risk factors for Creutzfeldt-Jakob disease.
  Journal
Neurobiol Dis 142:104973 (2020)
DOI:10.1016/j.nbd.2020.104973
Reference
PMID:18245809
  Authors
Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
  Journal
Am J Pathol 172:555-65 (2008)
DOI:10.2353/ajpath.2008.070442
Reference
PMID:15695097
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
  Journal
Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
PMID:17051207
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
  Journal
Nature 443:803-10 (2006)
DOI:10.1038/nature05294
Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
  Journal
Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
PMID:17085779
  Authors
Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
  Journal
J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
PMID:14705136
  Authors
Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
  Journal
J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
PMID:10398674
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
  Journal
Clin Microbiol Rev 12:429-44 (1999)
Reference
PMID:15890824
  Authors
Novakofski J, Brewer MS, Mateus-Pinilla N, Killefer J, McCusker RH
  Title
Prion biology relevant to bovine spongiform encephalopathy.
  Journal
J Anim Sci 83:1455-76 (2005)
DOI:10.2527/2005.8361455x
Reference
PMID:27581199
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
  Journal
Biochem Biophys Res Commun 483:1148-1155 (2017)
DOI:10.1016/j.bbrc.2016.07.118
Reference
PMID:31618844
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
  Journal
Int J Mol Sci 20:E5107 (2019)
DOI:10.3390/ijms20205107
Reference
PMID:31795242
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
  Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
  Journal
Int J Mol Sci 20:E6004 (2019)
DOI:10.3390/ijms20236004
Reference
PMID:30710214
  Authors
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
  Journal
Mol Neurobiol 56:6035-6045 (2019)
DOI:10.1007/s12035-019-1505-6
Reference
PMID:23439666
  Authors
Song Z, Zhao D, Yang L
  Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
  Journal
Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
PMID:28643372
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
PMID:29194372
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
  Journal
Pathogens 6:E63 (2017)
DOI:10.3390/pathogens6040063
Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
PMID:29476642
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
PMID:21722302
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
  Journal
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
Reference
PMID:18420416
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
  Journal
Neurobiol Dis 30:331-42 (2008)
DOI:10.1016/j.nbd.2008.02.003
Reference
PMID:16844381
  Authors
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
  Title
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
  Journal
Neurobiol Dis 23:669-78 (2006)
DOI:10.1016/j.nbd.2006.05.011
Reference
PMID:21980554
  Authors
Torres M, Encina G, Soto C, Hetz C
  Title
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
  Journal
Commun Integr Biol 4:258-61 (2011)
DOI:10.4161/cib.4.3.15019
Reference
PMID:25766616
  Authors
Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
  Journal
Exp Mol Med 47:e147 (2015)
DOI:10.1038/emm.2014.117
Reference
PMID:17466621
  Authors
Kristiansen M, Deriziotis P, Dimcheff DE, Jackson GS, Ovaa H, Naumann H, Clarke AR, van Leeuwen FW, Menendez-Benito V, Dantuma NP, Portis JL, Collinge J, Tabrizi SJ
  Title
Disease-associated prion protein oligomers inhibit the 26S proteasome.
  Journal
Mol Cell 26:175-88 (2007)
DOI:10.1016/j.molcel.2007.04.001
Reference
PMID:10195903
  Authors
Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
  Title
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
  Journal
Science 284:339-43 (1999)
DOI:10.1126/science.284.5412.339
Reference
PMID:14532116
  Authors
Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
  Title
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
  Journal
EMBO J 22:5435-45 (2003)
DOI:10.1093/emboj/cdg537
Reference
PMID:31417367
  Authors
Zamponi E, Pigino GF
  Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
  Journal
Front Cell Neurosci 13:350 (2019)
DOI:10.3389/fncel.2019.00350
Reference
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)
DOI:10.1371/journal.pone.0188340
Reference
PMID:21196165
  Authors
Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
  Title
Understanding the neurospecificity of Prion protein signaling.
  Journal
Front Biosci (Landmark Ed) 16:169-86 (2011)
DOI:10.2741/3682
Reference
PMID:23479001
  Authors
Didonna A
  Title
Prion protein and its role in signal transduction.
  Journal
Cell Mol Biol Lett 18:209-30 (2013)
DOI:10.2478/s11658-013-0085-0
Reference
PMID:28507517
  Authors
Shah SZA, Zhao D, Hussain T, Yang L
  Title
The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
  Journal
Front Aging Neurosci 9:120 (2017)
DOI:10.3389/fnagi.2017.00120
Reference
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.
  Journal
J Biol Chem 280:1529-34 (2005)
DOI:10.1074/jbc.M410966200
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.
  Journal
Proc Natl Acad Sci U S A 102:886-91 (2005)
DOI:10.1073/pnas.0408612101
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)
DOI:10.1186/1750-1326-5-6
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
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   
LinkDB

DBGET integrated database retrieval system