KEGG   PATHWAY: mlx05020
Entry
mlx05020                    Pathway                                
Name
Prion disease - Mirounga leonina (Southern elephant seal)
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
mlx05020  Prion disease
mlx05020

Disease
H00061  Prion disease
Organism
Mirounga leonina (Southern elephant seal) [GN:mlx]
Gene
117997521  [KO:K11352]
117997742  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
117997743  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
117997912  [KO:K02267]
117997916  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
117997961  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
117998137  KLC3; kinesin light chain 3 [KO:K10407]
117998200  [KO:K03938]
117998245  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
117998425  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
117998506  [KO:K02273]
117998533  [KO:K02272]
117998612  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
117998657  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
117998715  RAC2; ras-related C3 botulinum toxin substrate 2 isoform X1 [KO:K07860]
117998727  NCF4; neutrophil cytosol factor 4 isoform X1 [KO:K08012]
117998841  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
117998878  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
117999059  [KO:K03938]
117999128  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
117999153  [KO:K00415]
117999192  [KO:K03945]
117999338  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
117999511  NCF1; neutrophil cytosol factor 1 [KO:K08011]
117999517  [KO:K02138]
117999554  [KO:K02266]
117999637  MAPK3; mitogen-activated protein kinase 3 isoform X1 [KO:K04371] [EC:2.7.11.24]
117999772  [KO:K03061]
117999803  [KO:K07374]
117999807  [KO:K07374]
117999826  MAPK1; mitogen-activated protein kinase 1 isoform X1 [KO:K04371] [EC:2.7.11.24]
117999966  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
118000306  [KO:K03948]
118000336  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
118000345  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
118000514  [KO:K02131]
118000847  [KO:K02128]
118000958  [KO:K03949]
118001023  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
118001049  [KO:K00236]
118001095  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
118001187  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
118001264  [KO:K02267]
118001265  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
118001326  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
118001468  [KO:K02270]
118001502  [KO:K02263]
118001601  [KO:K02137]
118001612  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
118001772  [KO:K02732] [EC:3.4.25.1]
118001885  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
118002128  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
118002313  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
118002372  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
118002576  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
118002671  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
118002675  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
118002818  [KO:K02730] [EC:3.4.25.1]
118002863  [KO:K02730] [EC:3.4.25.1]
118002938  [KO:K02266]
118003073  [KO:K02267]
118003100  [KO:K02264]
118003203  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
118003737  RYR3; ryanodine receptor 3 [KO:K04963]
118004098  [KO:K02137]
118004305  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
118004315  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
118004320  [KO:K03948]
118004416  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
118004424  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922] [EC:2.7.1.153]
118004524  [KO:K05862]
118004526  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
118005005  [KO:K03945]
118005048  [KO:K00414]
118005070  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha [KO:K04345] [EC:2.7.11.11]
118005105  TUBB4A; tubulin beta-4A chain [KO:K07375]
118005112  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
118005176  PRKCD; protein kinase C delta type isoform X1 [KO:K06068] [EC:2.7.11.13]
118005189  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
118005208  [KO:K05214]
118005236  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
118005260  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
118005319  CAV3; caveolin-3 [KO:K12959]
118005325  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
118005417  [KO:K02268]
118005738  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
118005812  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
118005924  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
118005969  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
118006000  [KO:K00420]
118006042  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
118006050  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
118006077  [KO:K03945]
118006183  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
118006277  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
118006335  [KO:K04000]
118006357  [KO:K03995]
118006423  [KO:K03996]
118006469  [KO:K04000]
118006556  PIK3CA; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922] [EC:2.7.1.153]
118006559  [KO:K03996]
118006570  [KO:K03995]
118006627  [KO:K03945]
118006687  [KO:K03937]
118006688  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
118006809  [KO:K03937]
118006897  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
118007053  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
118007057  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
118007814  [KO:K02270]
118007866  IL1A; interleukin-1 alpha isoform X1 [KO:K04383]
118007867  IL1B; interleukin-1 beta [KO:K04519]
118007879  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
118008037  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
118008215  [KO:K02265]
118008426  KIF5A; kinesin heavy chain [KO:K10396]
118008635  [KO:K02270]
118008672  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
118008679  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
118008699  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
118008780  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
118008882  [KO:K07374]
118008883  [KO:K07374]
118008885  TUBA1C; tubulin alpha-1C chain [KO:K07374]
118008941  [KO:K03947]
118008962  [KO:K00417]
118008972  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853]
118008977  [KO:K03967]
118009132  [KO:K00417]
118009146  [KO:K21421] [EC:1.-.-.-]
118009157  [KO:K03952]
118009264  [KO:K03957]
118009645  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
118009813  [KO:K02265]
118009891  [KO:K02272]
118009917  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
118010066  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
118010074  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
118010248  [KO:K02271]
118010255  [KO:K02273]
118010299  [KO:K02138]
118010368  [KO:K04440] [EC:2.7.11.24]
118010572  [KO:K02725] [EC:3.4.25.1]
118010624  [KO:K02725] [EC:3.4.25.1]
118011073  [KO:K03948]
118011275  PRNP; major prion protein [KO:K05634]
118011354  [KO:K02263]
118011365  CSNK2A1; casein kinase II subunit alpha isoform X1 [KO:K03097] [EC:2.7.11.1]
118011878  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
118011954  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
118012024  [KO:K07374]
118012101  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
118012110  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
118012157  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
118012240  [KO:K02128]
118012283  [KO:K08738]
118012306  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
118012308  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
118012351  [KO:K02267]
118012355  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
118012403  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
118012420  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
118012462  KIF5C; kinesin heavy chain [KO:K10396]
118012526  [KO:K15041]
118012585  [KO:K02132]
118012943  RYR2; ryanodine receptor 2 [KO:K04962]
118013136  [KO:K02272]
118013166  [KO:K04741] [EC:3.4.22.-]
118013193  [KO:K03947]
118013236  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
118013240  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
118013335  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
118013637  [KO:K02135]
118013652  [KO:K03948]
118013662  [KO:K02135]
118013723  [KO:K03948]
118013784  HSPA6; LOW QUALITY PROTEIN: heat shock 70 kDa protein 6 [KO:K03283]
118013832  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
118014015  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
118014021  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
118014190  [KO:K07374]
118014221  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857]
118014290  [KO:K02136]
118014371  LAMC1; laminin subunit gamma-1 [KO:K05635]
118014374  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
118014376  [KO:K02138]
118014377  [KO:K02272]
118014566  [KO:K02131]
118014709  [KO:K03064]
118014721  [KO:K00417]
118014830  [KO:K03035]
118015043  [KO:K00417]
118015199  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
118015299  [KO:K00413]
118015397  [KO:K02726] [EC:3.4.25.1]
118015405  [KO:K02271]
118015807  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
118015836  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
118015844  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
118015981  [KO:K02273]
118015985  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
118016000  BAD; bcl2-associated agonist of cell death [KO:K02158]
118016083  KLC2; kinesin light chain 2 [KO:K10407]
118016140  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
118016147  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
118016235  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
118016240  [KO:K02128]
118016431  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
118016456  TUBB6; tubulin beta-6 chain [KO:K07375]
118016476  [KO:K03952]
118016628  [KO:K00418]
118016634  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
118016686  EGR1; early growth response protein 1 [KO:K09203]
118016727  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
118016863  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha isoform X1 [KO:K02649]
118016969  [KO:K02272]
118017147  [KO:K02138]
118017467  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
118017483  TUBB4B; tubulin beta-4B chain [KO:K07375]
118017502  C8G; complement component C8 gamma chain isoform X1 [KO:K03999]
118017516  NOTCH1; neurogenic locus notch homolog protein 1 isoform X1 [KO:K02599]
118017566  [KO:K03948]
118017681  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
118017695  PSMB7; proteasome subunit beta type-7 isoform X1 [KO:K02739] [EC:3.4.25.1]
118017734  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
118017747  C5; complement C5 [KO:K03994]
118017873  GRIN3A; glutamate receptor ionotropic, NMDA 3A [KO:K05213]
118017875  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
118017967  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
118018036  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
118018060  [KO:K02131]
118018155  [KO:K04392]
118018172  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
118018221  [KO:K02737] [EC:3.4.25.1]
118018432  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
118018546  [KO:K02266]
118018636  [KO:K00419]
118018772  NCAM2; neural cell adhesion molecule 2 [KO:K06491]
118018787  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
118018817  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
118018842  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
118019006  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
118019037  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
118019227  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
118019306  [KO:K02131]
118019364  C8B; complement component C8 beta chain [KO:K03998]
118019464  [KO:K00416]
118019468  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649]
118019589  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
118019630  [KO:K08738]
118019701  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
118019784  [KO:K02131]
118019908  C1QB; complement C1q subcomponent subunit B [KO:K03987]
118019909  C1QC; complement C1q subcomponent subunit C [KO:K03988]
118019910  C1QA; complement C1q subcomponent subunit A [KO:K03986]
118019915  [KO:K03948]
118019953  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
118019979  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
118020028  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
118020219  C8A; complement component C8 alpha chain [KO:K03997]
118020648  [KO:K03283]
118020657  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
118020809  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
118020828  [KO:K02131]
118020856  IL6; interleukin-6 [KO:K05405]
118020883  [KO:K08738]
118020909  [KO:K02131]
118020955  [KO:K02725] [EC:3.4.25.1]
118021007  CAV2; caveolin-2 [KO:K12958]
118021009  CAV1; caveolin-1 isoform X1 [KO:K06278]
118021036  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
118021108  [KO:K02128]
118021134  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
118021161  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
118021299  CREB5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
118021306  [KO:K02725] [EC:3.4.25.1]
118021333  [KO:K03937]
118021393  [KO:K02725] [EC:3.4.25.1]
118021446  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
118021556  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118021601  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
118021622  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
118021712  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
118021822  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
118021844  [KO:K02131]
118021900  [KO:K03948]
118021936  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
118022080  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118022199  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
118022312  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
118022338  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
118022663  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
118022700  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
118022759  [KO:K00411] [EC:7.1.1.8]
118022771  RYR1; ryanodine receptor 1 [KO:K04961]
118022776  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
118022877  [KO:K02267]
118022879  [KO:K02270]
118022951  [KO:K02725] [EC:3.4.25.1]
118023143  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
118023337  [KO:K02128]
118023396  [KO:K07375]
118023475  [KO:K08009]
118023493  [KO:K02263]
118023545  [KO:K02273]
118023608  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
118023729  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
118023788  [KO:K03967]
118024014  TUBB1; tubulin beta-1 chain [KO:K07375]
118024015  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
118024028  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
118024030  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
118024289  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
118024295  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
118024353  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
118024383  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
118024545  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
118024594  [KO:K03945]
118024680  [KO:K02730] [EC:3.4.25.1]
118024792  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
118024828  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
118024848  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
118025000  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
118025020  CCL5; C-C motif chemokine 5 [KO:K12499]
118025026  [KO:K02268]
118025330  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
118025455  [KO:K03957]
118025560  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
118025623  [KO:K02725] [EC:3.4.25.1]
118025638  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
118025758  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
118025809  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850]
118025829  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
118025936  [KO:K02273]
118025972  [KO:K03948]
118026049  [KO:K02271]
118026254  [KO:K03965]
118026286  [KO:K03947]
118026298  [KO:K02138]
118026484  [KO:K02128]
118026506  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
118026668  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
118026972  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
118027044  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118027255  TNF; tumor necrosis factor [KO:K03156]
118027278  [KO:K03938]
118027308  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta isoform X1 [KO:K09049]
118027322  HSPA1L; heat shock 70 kDa protein 1-like isoform X1 [KO:K03283]
118027334  CSNK2B; casein kinase II subunit beta isoform X1 [KO:K03115]
118027362  TUBB; tubulin beta chain [KO:K07375]
118027399  [KO:K07375]
118027402  TUBB2B; tubulin beta-2B chain [KO:K07375]
4355864  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
4355865  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
4355866  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
4355867  COX2; cytochrome c oxidase subunit II [KO:K02261]
4355868  COX3; cytochrome c oxidase subunit III [KO:K02262]
4355869  CYTB; cytochrome b [KO:K00412]
4355870  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
4355871  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
4355872  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
4355873  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
4355874  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
4355875  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
4355876  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
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Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
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Reference
PMID:25584786
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Goold R, McKinnon C, Tabrizi SJ
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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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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
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Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
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Reference
PMID:17085779
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Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
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J Mol Neurosci 29:195-214 (2006)
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Reference
PMID:14705136
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  Title
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
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  Title
Prion biology relevant to bovine spongiform encephalopathy.
  Journal
J Anim Sci 83:1455-76 (2005)
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Reference
PMID:27581199
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
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
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
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Int J Mol Sci 20:E5107 (2019)
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Reference
PMID:31795242
  Authors
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  Title
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
  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.
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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 6:63 (2017)
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Reference
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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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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
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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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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
PMID:17466621
  Authors
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  Title
Disease-associated prion protein oligomers inhibit the 26S proteasome.
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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.
  Title
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Science 284:339-43 (1999)
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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.
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EMBO J 22:5435-45 (2003)
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Reference
PMID:31417367
  Authors
Zamponi E, Pigino GF
  Title
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
  Title
Understanding the neurospecificity of Prion protein signaling.
  Journal
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.
  Journal
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.
  Journal
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.
  Journal
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)
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
mlx00190  Oxidative phosphorylation
mlx03050  Proteasome
mlx04020  Calcium signaling pathway
mlx04140  Autophagy - animal
mlx04141  Protein processing in endoplasmic reticulum
mlx04142  Lysosome
mlx04144  Endocytosis
mlx04210  Apoptosis
mlx04330  Notch signaling pathway
mlx04610  Complement and coagulation cascades
mlx04668  TNF signaling pathway
KO pathway
ko05020   

DBGET integrated database retrieval system