KEGG   PATHWAY: oga05020
Entry
oga05020                    Pathway                                
Name
Prion disease - Otolemur garnettii (small-eared galago)
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
oga05020  Prion disease
oga05020

Organism
Otolemur garnettii (small-eared galago) [GN:oga]
Gene
100959325  PRNP; major prion protein [KO:K05634]
100950780  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
100966590  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
100953638  LAMC1; laminin subunit gamma-1 [KO:K05635]
100957400  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
100948748  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100952149  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
100961044  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
100964083  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
100966533  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
100957285  BAX; apoptosis regulator BAX [KO:K02159]
100954541  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
100963635  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X2 [KO:K05208]
100943201  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
100966817  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100955117  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
100962947  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
100959828  GRIN3A; glutamate receptor ionotropic, NMDA 3A [KO:K05213]
100962049  GRIN3B; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
100957337  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
100964267  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
100953767  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X2 [KO:K04851]
100962519  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
100962683  CACNA1S; LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
100946481  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
100945512  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
100966174  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100940993  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
100956595  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
100962804  RYR1; ryanodine receptor 1 isoform X1 [KO:K04961]
100962105  RYR2; ryanodine receptor 2 [KO:K04962]
100956289  RYR3; ryanodine receptor 3 [KO:K04963]
100964437  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
100954512  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
100942290  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
100954053  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
100961754  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
100961025  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
100945846  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X2 [KO:K04348] [EC:3.1.3.16]
105886540  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
105887331  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
100962306  BAD; bcl2-associated agonist of cell death [KO:K02158]
100948868  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100962937  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
100945532  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
100942104  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100959761  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100958276  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
100945918  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100960533  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
100959812  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
100952744  cytochrome c, somatic [KO:K08738]
100946237  cytochrome c [KO:K08738]
100961637  cytochrome c, testis-specific [KO:K08738]
100951508  cytochrome c-like [KO:K08738]
100957904  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
100961435  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100950265  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
100964599  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100942143  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100966177  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944]
111722776  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
111723421  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
111724308  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
111725086  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
111727526  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100952131  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105888171  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111722661  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100951984  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100940452  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100946430  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100940934  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100953933  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
105887348  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100962441  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100960301  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100942336  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X2 [KO:K03954]
100942324  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100940967  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
100941628  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100941484  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
105886827  NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100943147  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100950170  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100951164  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100952107  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
100964200  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
100961354  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100947647  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
100956958  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X2 [KO:K03965]
100954930  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100951085  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
100952930  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial-like [KO:K11351]
100940365  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
100949204  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
100941289  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100948568  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
100948138  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100966743  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100965387  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
100962918  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100944722  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100948336  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100962647  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X2 [KO:K00234] [EC:1.3.5.1]
100965354  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
100960137  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
100963755  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
100949704  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
100953005  cytochrome c1, heme protein, mitochondrial [KO:K00413]
100947724  cytochrome b-c1 complex subunit 1, mitochondrial isoform X2 [KO:K00414]
100962593  cytochrome b-c1 complex subunit 2, mitochondrial isoform X1 [KO:K00415]
100954158  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
100941812  cytochrome b-c1 complex subunit 7 [KO:K00417]
100951496  cytochrome b-c1 complex subunit 8 isoform X1 [KO:K00418]
100950415  cytochrome b-c1 complex subunit 9 [KO:K00419]
100966663  cytochrome b-c1 complex subunit 10 [KO:K00420]
100964630  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
100944697  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
100952759  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
100948614  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
100950124  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
100957909  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
100956638  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
100949799  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100941254  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100949257  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
100955357  cytochrome c oxidase subunit 6B1 [KO:K02267]
100960658  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
111723020  cytochrome c oxidase subunit 6C-like [KO:K02268]
111723144  cytochrome c oxidase subunit 6C-like [KO:K02268]
111723145  cytochrome c oxidase subunit 6C-like [KO:K02268]
111723601  cytochrome c oxidase subunit 6C-like [KO:K02268]
111723999  cytochrome c oxidase subunit 6C-like [KO:K02268]
111724013  cytochrome c oxidase subunit 6C-like [KO:K02268]
111724125  cytochrome c oxidase subunit 6C-like [KO:K02268]
111724293  cytochrome c oxidase subunit 6C-like [KO:K02268]
111724885  cytochrome c oxidase subunit 6C-like [KO:K02268]
111725589  cytochrome c oxidase subunit 6C-like [KO:K02268]
111726159  cytochrome c oxidase subunit 6C [KO:K02268]
111726612  cytochrome c oxidase subunit 6C-like [KO:K02268]
111726804  cytochrome c oxidase subunit 6C-like [KO:K02268]
111726988  cytochrome c oxidase subunit 6C-like [KO:K02268]
111727107  cytochrome c oxidase subunit 6C-like [KO:K02268]
111727402  cytochrome c oxidase subunit 6C-like [KO:K02268]
111727426  cytochrome c oxidase subunit 6C-like [KO:K02268]
111727441  cytochrome c oxidase subunit 6C-like [KO:K02268]
111728416  cytochrome c oxidase subunit 6C-like [KO:K02268]
111728417  cytochrome c oxidase subunit 6C-like [KO:K02268]
100963028  cytochrome c oxidase subunit 6C [KO:K02268]
100954120  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
100957462  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100951390  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100960094  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100962771  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100951826  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100965534  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100958320  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100953844  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111723928  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111728562  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111728090  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
100944367  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100945994  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100962241  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
100964453  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100942284  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
100963316  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100945127  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100953993  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
100966365  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100951333  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
100955222  ATP synthase subunit d, mitochondrial [KO:K02138]
105887731  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100948313  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
100948751  PSMA2; proteasome subunit alpha type-2 isoform X2 [KO:K02726] [EC:3.4.25.1]
100941101  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
100952583  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
100945247  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100947299  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100960814  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100960922  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
100956915  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100943621  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
105886609  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100941738  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100964077  LOW QUALITY PROTEIN: proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
100953758  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100960749  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100959032  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100942655  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100957481  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100944801  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
100963717  PSMC1; LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4 [KO:K03062]
100961600  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100963843  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
100965975  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
100943523  PSMC5; 26S proteasome regulatory subunit 8 isoform X2 [KO:K03066]
100945869  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100965008  PSMD1; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100952064  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100961345  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
100966239  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100950546  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100952591  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
100955990  26S proteasome non-ATPase regulatory subunit 7-like [KO:K03038]
100951878  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100962652  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100944351  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
100941685  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
100960187  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100947028  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
100961050  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
100961815  C5; complement C5 isoform X1 [KO:K03994]
100965204  C6; complement component C6 [KO:K03995]
100948835  C7; complement component C7 [KO:K03996]
100963210  C8A; complement component C8 alpha chain [KO:K03997]
100963531  C8B; complement component C8 beta chain isoform X1 [KO:K03998]
100950742  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
100962993  C9; complement component C9 [KO:K04000]
100945458  heat shock 70 kDa protein 1 [KO:K03283]
100959513  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
100956095  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
100945147  HSPA1L; heat shock 70 kDa protein 1-like [KO:K03283]
100946722  heat shock 70 kDa protein 1 [KO:K03283]
100948116  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
100948513  CAV1; caveolin-1 [KO:K06278]
100961631  CAV2; caveolin-2 isoform X1 [KO:K12958]
100960553  CAV3; caveolin-3 [KO:K12959]
100947138  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
100953367  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922] [EC:2.7.1.153]
100954109  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
100956838  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta isoform X1 [KO:K02649]
100952580  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X2 [KO:K02649]
100949858  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
100951664  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
100951499  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
100958549  cytochrome b-245 light chain [KO:K08009]
100960840  NCF4; neutrophil cytosol factor 4 [KO:K08012]
100946950  NCF1; neutrophil cytosol factor 1 [KO:K08011]
100951845  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
100951369  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
100957182  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
100956269  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
100957118  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
100963993  MAPK11; mitogen-activated protein kinase 11 isoform X2 [KO:K04441] [EC:2.7.11.24]
100964304  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
100957169  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100967053  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100960275  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
100951533  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
100957343  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
100964324  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X2 [KO:K05870]
100966891  ATF2; cyclic AMP-dependent transcription factor ATF-2 [KO:K04450]
100966324  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
100951671  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
100941510  CREB3; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100955062  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
100952085  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100952718  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
100951769  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X2 [KO:K09049]
100961896  EGR1; early growth response protein 1 [KO:K09203]
100957034  CCL5; C-C motif chemokine 5 [KO:K12499]
100961897  IL1A; interleukin-1 alpha [KO:K04383]
100944877  IL1B; interleukin-1 beta [KO:K04519]
100949231  IL6; LOW QUALITY PROTEIN: interleukin-6 [KO:K05405]
100963613  TNF; tumor necrosis factor [KO:K03156]
100952751  GSK3B; glycogen synthase kinase-3 beta [KO:K03083] [EC:2.7.11.26]
100949419  CSNK2A1; LOW QUALITY PROTEIN: casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
100950929  CSNK2A2; casein kinase II subunit alpha' isoform X1 [KO:K03097] [EC:2.7.11.1]
100946935  KIF5A; kinesin heavy chain [KO:K10396]
100946006  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
100957718  KIF5B; kinesin-1 heavy chain [KO:K10396]
100958066  KLC4; kinesin light chain 4 [KO:K10407]
100953912  KLC2; kinesin light chain 2 isoform X2 [KO:K10407]
100963982  KLC3; kinesin light chain 3 [KO:K10407]
100954622  KLC1; kinesin light chain 1 isoform X9 [KO:K10407]
100947302  tubulin alpha-1B chain-like [KO:K07374]
100963795  tubulin alpha-3 chain-like [KO:K07374]
100967214  TUBA8; tubulin alpha-8 chain [KO:K07374]
100941549  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
100963937  LOW QUALITY PROTEIN: tubulin alpha chain-like [KO:K07374]
100946318  tubulin alpha-1A chain [KO:K07374]
100954785  tubulin alpha-3 chain-like [KO:K07374]
100942888  TUBA4A; tubulin alpha-4A chain isoform X1 [KO:K07374]
100945316  tubulin beta-3 chain [KO:K07375]
100944974  TUBB4A; tubulin beta-4A chain isoform X10 [KO:K07375]
100950590  tubulin beta chain-like [KO:K07375]
100964834  TUBB6; tubulin beta-6 chain [KO:K07375]
105887445  TUBB4B; tubulin beta-4B chain [KO:K07375]
100951977  TUBB; tubulin beta chain [KO:K07375]
100941657  TUBB1; tubulin beta-1 chain isoform X2 [KO:K07375]
100946820  TUBB2B; tubulin beta-2B 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.
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Expert Rev Mol Med 18:e5 (2016)
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Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
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Prion degradation pathways: Potential for therapeutic intervention.
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Mol Cell Neurosci 66:12-20 (2015)
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Reference
PMID:20889378
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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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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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Reference
PMID:15695097
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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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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
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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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Neuroprotective functions of prion protein.
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J Neurosci Res 75:153-61 (2004)
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Reference
PMID:10398674
  Authors
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Cellular biology of prion diseases.
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Reference
PMID:15890824
  Authors
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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Int J Mol Sci 20:E5107 (2019)
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Reference
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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
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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
  Authors
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
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  Authors
Hughes D, Mallucci GR
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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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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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Reference
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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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  Authors
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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
  Authors
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
  Authors
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
  Authors
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
  Authors
Didonna A
  Title
Prion protein and its role in signal transduction.
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Cell Mol Biol Lett 18:209-30 (2013)
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Reference
PMID:28507517
  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.
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Front Aging Neurosci 9:120 (2017)
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Reference
PMID:15528202
  Authors
Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
  Title
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
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J Biol Chem 280:1529-34 (2005)
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Reference
PMID:15640354
  Authors
Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
  Title
Notch-1 activation and dendritic atrophy in prion disease.
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Proc Natl Acad Sci U S A 102:886-91 (2005)
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Reference
PMID:20205843
  Authors
Dearmond SJ, Bajsarowicz K
  Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
  Journal
Mol Neurodegener 5:6 (2010)
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Reference
PMID:29998397
  Authors
Hirsch TZ, Martin-Lanneree S, Reine F, Hernandez-Rapp J, Herzog L, Dron M, Privat N, Passet B, Halliez S, Villa-Diaz A, Lacroux C, Klein V, Haik S, Andreoletti O, Torres JM, Vilotte JL, Beringue V, Mouillet-Richard S
  Title
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases.
  Journal
Mol Neurobiol 56:2159-2173 (2019)
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Reference
PMID:28714865
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
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
oga00190  Oxidative phosphorylation
oga03050  Proteasome
oga04020  Calcium signaling pathway
oga04140  Autophagy - animal
oga04141  Protein processing in endoplasmic reticulum
oga04142  Lysosome
oga04144  Endocytosis
oga04210  Apoptosis
oga04330  Notch signaling pathway
oga04610  Complement and coagulation cascades
oga04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

DBGET integrated database retrieval system