KEGG   PATHWAY: mbez05020
Entry
mbez05020                   Pathway                                
Name
Prion disease - Moschus berezovskii (Chinese forest musk deer)
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
mbez05020  Prion disease
mbez05020

Organism
Moschus berezovskii (Chinese forest musk deer) [GN:mbez]
Gene
129559932  PRNP; major prion protein [KO:K05634]
129553178  NCAM2; neural cell adhesion molecule 2 isoform X1 [KO:K06491]
129554339  NCAM1; neural cell adhesion molecule 1 isoform X1 [KO:K06491]
129551306  LAMC1; laminin subunit gamma-1 [KO:K05635]
129552765  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
129548827  STIP1; stress-induced-phosphoprotein 1 [KO:K09553]
129558401  PRKACA; cAMP-dependent protein kinase catalytic subunit alpha isoform X1 [KO:K04345] [EC:2.7.11.11]
129540637  PRKACB; cAMP-dependent protein kinase catalytic subunit beta isoform X1 [KO:K04345] [EC:2.7.11.11]
129546572  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
129561305  FYN; tyrosine-protein kinase Fyn isoform X1 [KO:K05703] [EC:2.7.10.2]
129539066  GRIN1; glutamate receptor ionotropic, NMDA 1 [KO:K05208]
129545368  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
129536255  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
129538079  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
129546598  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
129558929  GRIN3B; glutamate receptor ionotropic, NMDA 3B [KO:K05214]
129539723  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
129535727  CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850]
129547932  CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851]
129564275  CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853]
129551192  CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857]
129539411  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
129539222  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
129562722  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 isoform X1 [KO:K03237]
129536687  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
129540547  LOW QUALITY PROTEIN: cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
129537221  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
129546860  RYR1; ryanodine receptor 1 isoform X1 [KO:K04961]
129555843  RYR2; ryanodine receptor 2 [KO:K04962]
129562454  RYR3; ryanodine receptor 3 isoform X1 [KO:K04963]
129548039  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
129536224  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959]
129543844  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
129556888  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
129562133  PPP3CA; protein phosphatase 3 catalytic subunit alpha isoform X1 [KO:K04348] [EC:3.1.3.16]
129555711  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
129539040  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
129548816  BAD; bcl2-associated agonist of cell death [KO:K02158]
129558217  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
129555687  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
129549434  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
129560157  voltage-dependent anion-selective channel protein 3-like [KO:K15041]
129562882  voltage-dependent anion-selective channel protein 3-like [KO:K15041]
129542617  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
129549309  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
129564629  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
129535024  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
129555671  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
129535887  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
129536239  cytochrome c-like [KO:K08738]
129538389  cytochrome c 2 [KO:K08738]
129552526  cytochrome c [KO:K08738]
129551377  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
129549305  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
129555725  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
7872555  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
7872543  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
7872549  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
7872551  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
7872550  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
7872552  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
7872553  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
129548685  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
129549785  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
129548146  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
129559361  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
129546048  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947]
129536721  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
129536909  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
129552258  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
129557319  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
129552607  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
129536257  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
129558629  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
129539677  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
129536388  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
129540843  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
129545460  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
129559417  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
129536626  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
129559529  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
129552713  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
129538372  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
129562206  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
129557517  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
129558310  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
129563161  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
129563333  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
129538417  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
129555441  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
129539436  NDUFB9; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
129545250  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
129564391  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
129537781  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
129541195  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
129554830  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
129553266  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
129541431  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
129553392  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
129558789  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
129548680  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
129542435  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
129549039  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
129553417  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
129537568  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
129540820  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
129551518  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
129554330  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
129547289  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
7872554  CYTB; cytochrome b [KO:K00412]
129560239  cytochrome c1, heme protein, mitochondrial [KO:K00413]
129547809  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
129545439  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
129541300  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
129538687  cytochrome b-c1 complex subunit 7-like [KO:K00417]
129559837  cytochrome b-c1 complex subunit 7 [KO:K00417]
129538008  cytochrome b-c1 complex subunit 8-like [KO:K00418]
129559505  cytochrome b-c1 complex subunit 8 [KO:K00418]
7872548  COX3; cytochrome c oxidase subunit III [KO:K02262]
7872544  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
7872545  COX2; cytochrome c oxidase subunit II [KO:K02261]
129547198  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
129562077  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
129550450  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
129539072  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
129539786  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
129545568  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
129542288  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
129546050  cytochrome c oxidase subunit 6B2 [KO:K02267]
129546145  cytochrome c oxidase subunit 6B1 [KO:K02267]
129539783  cytochrome c oxidase subunit 6B1-like [KO:K02267]
129559906  cytochrome c oxidase subunit 6C [KO:K02268]
129537105  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
129546136  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
129560985  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
129534867  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
129550158  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
129549821  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
129535664  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
129549267  LOW QUALITY PROTEIN: ATP synthase subunit beta, mitochondrial-like [KO:K02133] [EC:7.1.2.2]
129556910  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
129558473  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
7872547  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
129536251  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
129541042  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
129536222  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
129544287  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
129538226  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
129544741  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
129545120  ATP synthase subunit d, mitochondrial-like [KO:K02138]
129548151  ATP synthase subunit d, mitochondrial-like [KO:K02138]
129552488  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
7872546  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
129550517  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
129552575  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
129550408  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
129549718  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
129561363  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
129541114  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
129554486  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
129562420  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
129562506  proteasome subunit alpha type-3-like [KO:K02727] [EC:3.4.25.1]
129537072  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
129539899  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
129544336  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
129540914  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
129562452  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
129560868  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
129540647  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
129552369  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
129562511  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
129546821  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
129562650  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
129554823  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
129544542  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
129545229  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
129538585  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
129544367  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
129542628  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
129544562  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
129540095  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
129547987  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
129546950  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
129548583  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
129549582  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 4-like [KO:K03029]
129548374  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 4-like [KO:K03029]
129537555  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
129546868  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
129561284  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
129557216  C5; complement C5 [KO:K03994]
129553056  C6; complement component C6 [KO:K03995]
129553094  C7; complement component C7 [KO:K03996]
129541700  C8A; complement component C8 alpha chain [KO:K03997]
129541518  C8B; complement component C8 beta chain [KO:K03998]
129539174  C8G; complement component C8 gamma chain [KO:K03999]
129553303  C9; complement component C9 [KO:K04000]
129537823  C1QA; complement C1q subcomponent subunit A [KO:K03986]
129538397  C1QB; complement C1q subcomponent subunit B [KO:K03987]
129538391  C1QC; complement C1q subcomponent subunit C [KO:K03988]
129543099  heat shock 70 kDa protein 1-like [KO:K03283]
129543100  heat shock 70 kDa protein 1B [KO:K03283]
129556947  heat shock cognate 71 kDa protein-like [KO:K03283]
129540977  HSPA6; heat shock 70 kDa protein 6 [KO:K03283]
129554454  HSPA8; heat shock cognate 71 kDa protein [KO:K03283]
129539195  NOTCH1; neurogenic locus notch homolog protein 1 [KO:K02599]
129552427  CAV1; caveolin-1 isoform X1 [KO:K06278]
129552428  CAV2; caveolin-2 [KO:K12958]
129548045  CAV3; caveolin-3 [KO:K12959]
129551508  PIK3CD; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
129547146  PIK3CB; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform isoform X1 [KO:K00922] [EC:2.7.1.153]
129559156  PIK3R2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649]
129553207  PIK3R1; phosphatidylinositol 3-kinase regulatory subunit alpha [KO:K02649]
129540801  PIK3R3; phosphatidylinositol 3-kinase regulatory subunit gamma isoform X1 [KO:K02649]
129547928  PRKCD; protein kinase C delta type [KO:K06068] [EC:2.7.11.13]
129564517  cytochrome b-245 heavy chain [KO:K21421] [EC:1.-.-.-]
129547179  cytochrome b-245 light chain [KO:K08009]
129536900  NCF4; neutrophil cytosol factor 4 [KO:K08012]
129545605  NCF1; neutrophil cytosol factor 1 isoform X1 [KO:K08011]
129551303  NCF2; neutrophil cytosol factor 2 isoform X1 [KO:K08010]
129545763  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
129539810  ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
129554914  ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
129536787  RAC2; ras-related C3 botulinum toxin substrate 2 [KO:K07860]
129537071  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
129537073  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
129543604  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
129543888  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
129545108  mitogen-activated protein kinase 14-like [KO:K04441] [EC:2.7.11.24]
129559467  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
129561625  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
129555633  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
129542485  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
129545501  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
129538457  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
129537505  ATF2; cyclic AMP-dependent transcription factor ATF-2 isoform X1 [KO:K04450]
129556793  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
129559023  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
129552685  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
129540564  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
129554794  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
129552502  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
129543841  ATF6B; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049]
129559101  EGR1; early growth response protein 1 [KO:K09203]
129536712  CCL5; C-C motif chemokine 5 [KO:K12499]
129539790  IL1A; interleukin-1 alpha [KO:K04383]
129539906  IL1B; interleukin-1 beta [KO:K04519]
129543262  TNF; tumor necrosis factor isoform X1 [KO:K03156]
129561928  GSK3B; glycogen synthase kinase-3 beta isoform X1 [KO:K03083] [EC:2.7.11.26]
129547060  CSNK2A2; casein kinase II subunit alpha' [KO:K03097] [EC:2.7.11.1]
129561950  CSNK2A1; casein kinase II subunit alpha [KO:K03097] [EC:2.7.11.1]
129543628  CSNK2B; casein kinase II subunit beta [KO:K03115]
129535944  KIF5A; kinesin heavy chain [KO:K10396]
129558468  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
129538351  KIF5C; kinesin heavy chain [KO:K10396]
129543512  KLC4; kinesin light chain 4 [KO:K10407]
129550693  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
129546675  KLC3; kinesin light chain 3 [KO:K10407]
129548736  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
129536409  TUBA8; tubulin alpha-8 chain [KO:K07374]
129536741  LOW QUALITY PROTEIN: tubulin alpha-1B chain-like [KO:K07374]
129536760  tubulin alpha-1A chain isoform X1 [KO:K07374]
129536761  tubulin alpha-1C chain isoform X1 [KO:K07374]
129536762  tubulin alpha-1C chain-like isoform X1 [KO:K07374]
129537982  tubulin alpha-4A chain [KO:K07374]
129538092  tubulin alpha-1D chain isoform X1 [KO:K07374]
129554739  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
129543045  tubulin beta-4B chain-like [KO:K07375]
129543147  TUBB; tubulin beta chain [KO:K07375]
129543421  tubulin beta-2B chain [KO:K07375]
129549800  TUBB6; tubulin beta-6 chain [KO:K07375]
129543715  TUBB2A; tubulin beta-2A chain [KO:K07375]
129558972  tubulin beta chain-like [KO:K07375]
129559232  TUBB4A; tubulin beta-4A chain [KO:K07375]
129539645  TUBB4B; tubulin beta-4B chain [KO:K07375]
129561483  TUBB1; tubulin beta-1 chain [KO:K07375]
129547355  uncharacterized protein LOC129547355 [KO:K07375]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
  Authors
Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
DOI:10.1017/erm.2016.8
Reference
  Authors
Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
  Journal
Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
Reference
  Authors
Soto C, Satani N
  Title
The intricate mechanisms of neurodegeneration in prion diseases.
  Journal
Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
  Authors
Mays CE, Soto C
  Title
The stress of prion disease.
  Journal
Brain Res 1648:553-560 (2016)
DOI:10.1016/j.brainres.2016.04.009
Reference
  Authors
Jones E, Mead S
  Title
Genetic risk factors for Creutzfeldt-Jakob disease.
  Journal
Neurobiol Dis 142:104973 (2020)
DOI:10.1016/j.nbd.2020.104973
Reference
  Authors
Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
  Journal
Am J Pathol 172:555-65 (2008)
DOI:10.2353/ajpath.2008.070442
Reference
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
  Journal
Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
  Journal
Nature 443:803-10 (2006)
DOI:10.1038/nature05294
Reference
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
  Journal
Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
  Authors
Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
  Journal
J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
  Authors
Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
  Journal
J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
  Journal
Clin Microbiol Rev 12:429-44 (1999)
Reference
  Authors
Novakofski J, Brewer MS, Mateus-Pinilla N, Killefer J, McCusker RH
  Title
Prion biology relevant to bovine spongiform encephalopathy.
  Journal
J Anim Sci 83:1455-76 (2005)
DOI:10.2527/2005.8361455x
Reference
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
  Journal
Biochem Biophys Res Commun 483:1148-1155 (2017)
DOI:10.1016/j.bbrc.2016.07.118
Reference
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
  Journal
Int J Mol Sci 20:E5107 (2019)
DOI:10.3390/ijms20205107
Reference
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
  Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
  Journal
Int J Mol Sci 20:E6004 (2019)
DOI:10.3390/ijms20236004
Reference
  Authors
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
  Journal
Mol Neurobiol 56:6035-6045 (2019)
DOI:10.1007/s12035-019-1505-6
Reference
  Authors
Song Z, Zhao D, Yang L
  Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
  Journal
Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
  Journal
Pathogens 6:E63 (2017)
DOI:10.3390/pathogens6040063
Reference
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
  Journal
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
Reference
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
  Journal
Neurobiol Dis 30:331-42 (2008)
DOI:10.1016/j.nbd.2008.02.003
Reference
  Authors
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
  Title
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
  Journal
Neurobiol Dis 23:669-78 (2006)
DOI:10.1016/j.nbd.2006.05.011
Reference
  Authors
Torres M, Encina G, Soto C, Hetz C
  Title
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
  Journal
Commun Integr Biol 4:258-61 (2011)
DOI:10.4161/cib.4.3.15019
Reference
  Authors
Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
  Journal
Exp Mol Med 47:e147 (2015)
DOI:10.1038/emm.2014.117
Reference
  Authors
Kristiansen M, Deriziotis P, Dimcheff DE, Jackson GS, Ovaa H, Naumann H, Clarke AR, van Leeuwen FW, Menendez-Benito V, Dantuma NP, Portis JL, Collinge J, Tabrizi SJ
  Title
Disease-associated prion protein oligomers inhibit the 26S proteasome.
  Journal
Mol Cell 26:175-88 (2007)
DOI:10.1016/j.molcel.2007.04.001
Reference
  Authors
Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
  Title
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
  Journal
Science 284:339-43 (1999)
DOI:10.1126/science.284.5412.339
Reference
  Authors
Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
  Title
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
  Journal
EMBO J 22:5435-45 (2003)
DOI:10.1093/emboj/cdg537
Reference
  Authors
Zamponi E, Pigino GF
  Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
  Journal
Front Cell Neurosci 13:350 (2019)
DOI:10.3389/fncel.2019.00350
Reference
  Authors
Zamponi E, Buratti F, Cataldi G, Caicedo HH, Song Y, Jungbauer LM, LaDu MJ, Bisbal M, Lorenzo A, Ma J, Helguera PR, Morfini GA, Brady ST, Pigino GF
  Title
Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
  Journal
PLoS One 12:e0188340 (2017)
DOI:10.1371/journal.pone.0188340
Reference
  Authors
Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
  Title
Understanding the neurospecificity of Prion protein signaling.
  Journal
Front Biosci (Landmark Ed) 16:169-86 (2011)
DOI:10.2741/3682
Reference
  Authors
Didonna A
  Title
Prion protein and its role in signal transduction.
  Journal
Cell Mol Biol Lett 18:209-30 (2013)
DOI:10.2478/s11658-013-0085-0
Reference
  Authors
Shah SZA, Zhao D, Hussain T, Yang L
  Title
The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
  Journal
Front Aging Neurosci 9:120 (2017)
DOI:10.3389/fnagi.2017.00120
Reference
  Authors
Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
  Title
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
  Journal
J Biol Chem 280:1529-34 (2005)
DOI:10.1074/jbc.M410966200
Reference
  Authors
Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
  Title
Notch-1 activation and dendritic atrophy in prion disease.
  Journal
Proc Natl Acad Sci U S A 102:886-91 (2005)
DOI:10.1073/pnas.0408612101
Reference
  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
  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
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
  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
  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
mbez00190  Oxidative phosphorylation
mbez03050  Proteasome
mbez04020  Calcium signaling pathway
mbez04140  Autophagy - animal
mbez04141  Protein processing in endoplasmic reticulum
mbez04142  Lysosome
mbez04144  Endocytosis
mbez04210  Apoptosis
mbez04330  Notch signaling pathway
mbez04610  Complement and coagulation cascades
mbez04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

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