KEGG   PATHWAY: umr05020
Entry
umr05020                    Pathway                                
Name
Prion disease - Ursus maritimus (polar bear)
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
umr05020  Prion disease
umr05020

Disease
H00061  Prion disease
Organism
Ursus maritimus (polar bear) [GN:umr]
Gene
103656310  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
103656462  [KO:K02271]
103656470  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
103656532  KIF5B; kinesin-1 heavy chain [KO:K10396]
103656598  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
103656636  KIF5A; kinesin heavy chain [KO:K10396]
103656658  [KO:K00413]
103656991  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
103657024  [KO:K03945]
103657107  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
103657183  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
103657232  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
103657400  [KO:K10881]
103657485  [KO:K02270]
103657723  [KO:K07374]
103657769  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
103657827  MAPK3; mitogen-activated protein kinase 3 isoform X1 [KO:K04371] [EC:2.7.11.24]
103657893  [KO:K02266]
103657962  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
103658293  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
103658639  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
103658750  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
103658816  [KO:K02272]
103658820  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
103658871  IL6; interleukin-6 [KO:K05405]
103658902  [KO:K08738]
103658932  [KO:K09047]
103659249  [KO:K00419]
103659502  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
103659515  [KO:K03939]
103659593  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
103659737  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
103659788  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
103659802  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
103659841  KIF5C; kinesin heavy chain [KO:K10396]
103659901  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
103660109  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
103660136  APAF1; LOW QUALITY PROTEIN: apoptotic protease-activating factor 1 [KO:K02084]
103660313  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
103660341  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
103660584  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
103660617  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
103660779  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
103661007  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
103661036  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
103661069  RYR1; LOW QUALITY PROTEIN: ryanodine receptor 1 [KO:K04961]
103661161  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
103661190  [KO:K03948]
103661230  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
103661483  [KO:K00417]
103661502  [KO:K02268]
103661621  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
103662005  RYR2; ryanodine receptor 2 [KO:K04962]
103662209  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
103662283  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
103662415  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
103662441  [KO:K04741] [EC:3.4.22.-]
103662527  NCAM1; neural cell adhesion molecule 1 [KO:K06491]
103662680  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
103662744  [KO:K07374]
103662822  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
103662923  [KO:K08738]
103662972  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
103663617  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X3 [KO:K02649]
103663752  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
103663781  EGR1; early growth response protein 1 [KO:K09203]
103663786  [KO:K00416]
103663788  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
103663818  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
103663827  [KO:K00418]
103664073  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
103664628  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
103664673  C6; complement component C6 [KO:K03995]
103664674  C7; complement component C7 [KO:K03996]
103664686  C9; complement component C9 [KO:K04000]
103664901  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
103664961  TUBB2A; tubulin beta-2A chain isoform X1 [KO:K07375]
103665004  TUBB; tubulin beta chain [KO:K07375]
103665275  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
103665451  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
103665538  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
103665553  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
103665634  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
103665659  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
103665721  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
103665926  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
103666073  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
103666331  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
103666354  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
103666399  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
103666575  [KO:K03986]
103666614  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
103666628  CASP9; caspase-9 isoform X2 [KO:K04399] [EC:3.4.22.62]
103666874  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922] [EC:2.7.1.153]
103667024  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
103667087  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
103667294  CCL5; C-C motif chemokine 5 [KO:K12499]
103667431  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
103667513  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
103667517  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
103667625  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
103667825  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
103667846  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
103667893  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
103668021  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
103668033  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
103668219  [KO:K11352]
103668224  [KO:K03945]
103668228  [KO:K21421] [EC:1.-.-.-]
103668409  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
103668590  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
103668772  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
103668880  [KO:K00415]
103668899  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
103668940  [KO:K03037]
103669030  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
103669052  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
103669231  [KO:K07374]
103669237  [KO:K07374]
103669286  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
103669585  CAV3; caveolin-3 [KO:K12959]
103669597  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
103669950  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
103670095  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
103670166  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
103670323  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
103670329  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
103670332  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
103670360  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
103670372  C5; complement C5 [KO:K03994]
103670671  C8B; complement component C8 beta chain [KO:K03998]
103670672  C8A; complement component C8 alpha chain [KO:K03997]
103670755  [KO:K00416]
103670766  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649]
103670961  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
103670962  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
103671026  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
103671191  [KO:K02265]
103671340  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
103671495  IL1B; interleukin-1 beta isoform X2 [KO:K04519]
103671508  [KO:K03945]
103671542  [KO:K02270]
103671855  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
103671864  IL1A; interleukin-1 alpha [KO:K04383]
103671936  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
103672010  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
103672016  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
103672146  [KO:K02272]
103672304  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
103672308  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
103672393  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
103672508  NCF2; neutrophil cytosol factor 2 [KO:K08010]
103672511  LAMC1; laminin subunit gamma-1 [KO:K05635]
103672701  [KO:K02271]
103672884  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
103672921  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
103673070  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
103673102  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
103673122  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
103673149  [KO:K02266]
103673187  NCAM2; neural cell adhesion molecule 2 [KO:K06491]
103673318  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
103673361  TUBB6; tubulin beta-6 chain [KO:K07375]
103673363  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
103673560  RYR3; LOW QUALITY PROTEIN: ryanodine receptor 3 [KO:K04963]
103673708  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
103673753  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
103673805  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
103673823  [KO:K00411] [EC:7.1.1.8]
103673835  [KO:K02263]
103673846  [KO:K08009]
103673863  [KO:K07375]
103674004  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
103674068  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
103674115  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
103674226  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
103674244  [KO:K00417]
103674346  [KO:K02263]
103674361  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
103674536  [KO:K05634]
103674585  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
103674739  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
103674744  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
103674845  [KO:K00414]
103674915  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
103674995  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
103675040  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
103675162  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
103675247  C8G; complement component C8 gamma chain [KO:K03999]
103675253  TUBB4B; tubulin beta-4B chain [KO:K07375]
103675283  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
103675310  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
103675324  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
103675456  TUBA1B; tubulin alpha-1B chain [KO:K07374]
103675457  [KO:K07374]
103675459  [KO:K07374]
103675541  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
103675693  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
103675706  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
103675753  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
103675819  [KO:K02271]
103675859  [KO:K02267]
103676082  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
103676116  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X2 [KO:K03961]
103676125  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
103676131  [KO:K03948]
103676258  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X2 [KO:K02136]
103676280  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
103676372  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
103676407  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
103676418  NCF4; neutrophil cytosol factor 4 [KO:K08012]
103676584  CAV1; caveolin-1 isoform X1 [KO:K06278]
103676612  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
103676671  CAV2; caveolin-2 [KO:K12958]
103676885  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
103676947  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
103676988  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
103677056  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
103677527  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
103677737  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
103677743  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
103677769  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
103677943  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
103678138  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
103678327  [KO:K02266]
103678534  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
103678592  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
103678631  GRIN3A; glutamate receptor ionotropic, NMDA 3A [KO:K05213]
103678783  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
103678785  GSK3B; glycogen synthase kinase-3 beta isoform X2 [KO:K03083] [EC:2.7.11.26]
103679121  [KO:K02264]
103679235  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
103679246  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
103679251  [KO:K03947]
103679417  [KO:K02273]
103679423  BAD; bcl2-associated agonist of cell death [KO:K02158]
103679486  KLC2; kinesin light chain 2 [KO:K10407]
103679522  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
103679545  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
103679622  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
103679714  [KO:K02265]
103679757  [KO:K02732] [EC:3.4.25.1]
103679990  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
103680303  [KO:K02267]
103680708  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
103680991  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
103681007  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
103681035  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
103681631  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
103681635  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
103681641  [KO:K00420]
103681679  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
103681700  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
103681710  TUBB4A; tubulin beta-4A chain [KO:K07375]
103681725  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
103681771  GRIN3B; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
103682114  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
103682183  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
103682282  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
103682293  [KO:K03283]
103682294  [KO:K03283]
103682295  [KO:K03283]
103682308  CSNK2B; casein kinase II subunit beta [KO:K03115]
103682322  TNF; tumor necrosis factor [KO:K03156]
103682383  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
103682512  [KO:K02267]
103682538  [KO:K02270]
121102487  [KO:K03945]
121104237  [KO:K08738]
121106055  [KO:K03283]
121106099  [KO:K03037]
804861  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
804862  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
804863  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
804864  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
804865  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
804866  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
804867  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
804868  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
804869  COX3; cytochrome c oxidase subunit III [KO:K02262]
804870  COX2; cytochrome c oxidase subunit II [KO:K02261]
804871  CYTB; cytochrome b [KO:K00412]
804872  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
804873  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
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  Title
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Expert Rev Mol Med 18:e5 (2016)
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The highways and byways of prion protein trafficking.
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Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
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Prions and their partners in crime.
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Nature 443:803-10 (2006)
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Neurobiol Dis 8:743-63 (2001)
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J Anim Sci 83:1455-76 (2005)
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Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
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Biochem Biophys Res Commun 483:1148-1155 (2017)
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Prion Protein in Glioblastoma Multiforme.
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Int J Mol Sci 20:E5107 (2019)
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Int J Mol Sci 20:E6004 (2019)
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Mol Neurobiol 56:6035-6045 (2019)
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Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
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Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
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Fine-tuning PERK signaling for neuroprotection.
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J Neurochem 142:812-826 (2017)
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Hughes D, Halliday M
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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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Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
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Nat Rev Neurosci 15:233-49 (2014)
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J Cell Mol Med 15:2025-39 (2011)
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  Title
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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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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Mol Cell 26:175-88 (2007)
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Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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EMBO J 22:5435-45 (2003)
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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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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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Understanding the neurospecificity of Prion protein signaling.
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Front Biosci (Landmark Ed) 16:169-86 (2011)
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Prion protein and its role in signal transduction.
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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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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
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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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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
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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
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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
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Aguzzi A, Zhu C
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Microglia in prion diseases.
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J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
  Authors
Mabbott NA
  Title
The complement system in prion diseases.
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Curr Opin Immunol 16:587-93 (2004)
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Reference
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Mallucci G, Collinge J
  Title
Rational targeting for prion therapeutics.
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Nat Rev Neurosci 6:23-34 (2005)
DOI:10.1038/nrn1584
Related
pathway
umr00190  Oxidative phosphorylation
umr03050  Proteasome
umr04020  Calcium signaling pathway
umr04140  Autophagy - animal
umr04141  Protein processing in endoplasmic reticulum
umr04142  Lysosome
umr04144  Endocytosis
umr04210  Apoptosis
umr04330  Notch signaling pathway
umr04610  Complement and coagulation cascades
umr04668  TNF signaling pathway
KO pathway
ko05020   

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