KEGG   PATHWAY: mmu05020
Entry
mmu05020                    Pathway                                
Name
Prion disease - Mus musculus (house mouse)
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
mmu05020  Prion disease
mmu05020

Organism
Mus musculus (house mouse) [GN:mmu]
Gene
19122  Prnp; prion protein [KO:K05634]
17967  Ncam1; neural cell adhesion molecule 1 [KO:K06491]
17968  Ncam2; neural cell adhesion molecule 2 [KO:K06491]
226519  Lamc1; laminin, gamma 1 [KO:K05635]
20655  Sod1; superoxide dismutase 1, soluble [KO:K04565] [EC:1.15.1.1]
20867  Stip1; stress-induced phosphoprotein 1 [KO:K09553]
18747  Prkaca; protein kinase, cAMP dependent, catalytic, alpha [KO:K04345] [EC:2.7.11.11]
18749  Prkacb; protein kinase, cAMP dependent, catalytic, beta [KO:K04345] [EC:2.7.11.11]
12028  Bax; BCL2-associated X protein [KO:K02159]
14360  Fyn; Fyn proto-oncogene [KO:K05703] [EC:2.7.10.2]
14810  Grin1; glutamate receptor, ionotropic, NMDA1 (zeta 1) [KO:K05208]
14811  Grin2a; glutamate receptor, ionotropic, NMDA2A (epsilon 1) [KO:K05209]
14812  Grin2b; glutamate receptor, ionotropic, NMDA2B (epsilon 2) [KO:K05210]
14813  Grin2c; glutamate receptor, ionotropic, NMDA2C (epsilon 3) [KO:K05211]
14814  Grin2d; glutamate receptor, ionotropic, NMDA2D (epsilon 4) [KO:K05212]
242443  Grin3a; glutamate receptor ionotropic, NMDA3A [KO:K05213]
170483  Grin3b; glutamate receptor, ionotropic, NMDA3B [KO:K05214]
12287  Cacna1b; calcium channel, voltage-dependent, N type, alpha 1B subunit [KO:K04849]
12288  Cacna1c; calcium channel, voltage-dependent, L type, alpha 1C subunit [KO:K04850]
12289  Cacna1d; calcium channel, voltage-dependent, L type, alpha 1D subunit [KO:K04851]
54652  Cacna1f; calcium channel, voltage-dependent, alpha 1F subunit [KO:K04853]
12292  Cacna1s; calcium channel, voltage-dependent, L type, alpha 1S subunit [KO:K04857]
14828  Hspa5; heat shock protein 5 [KO:K09490] [EC:3.6.4.10]
13666  Eif2ak3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
13665  Eif2s1; eukaryotic translation initiation factor 2, subunit 1 alpha [KO:K03237]
11911  Atf4; activating transcription factor 4 [KO:K04374]
13198  Ddit3; DNA-damage inducible transcript 3 [KO:K04452]
20190  Ryr1; ryanodine receptor 1, skeletal muscle [KO:K04961]
20191  Ryr2; ryanodine receptor 2, cardiac [KO:K04962]
20192  Ryr3; ryanodine receptor 3 [KO:K04963]
16438  Itpr1; inositol 1,4,5-trisphosphate receptor 1 [KO:K04958]
16439  Itpr2; inositol 1,4,5-triphosphate receptor 2 [KO:K04959]
16440  Itpr3; inositol 1,4,5-triphosphate receptor 3 [KO:K04960]
12364  Casp12; caspase 12 [KO:K04741] [EC:3.4.22.-]
19057  Ppp3cc; protein phosphatase 3, catalytic subunit, gamma isoform [KO:K04348] [EC:3.1.3.16]
19055  Ppp3ca; protein phosphatase 3, catalytic subunit, alpha isoform [KO:K04348] [EC:3.1.3.16]
19056  Ppp3cb; protein phosphatase 3, catalytic subunit, beta isoform [KO:K04348] [EC:3.1.3.16]
19058  Ppp3r1; protein phosphatase 3, regulatory subunit B, alpha isoform (calcineurin B, type I) [KO:K06268]
19059  Ppp3r2; protein phosphatase 3, regulatory subunit B, alpha isoform (calcineurin B, type II) [KO:K06268]
12015  Bad; BCL2-associated agonist of cell death [KO:K02158]
22333  Vdac1; voltage-dependent anion channel 1 [KO:K05862]
22334  Vdac2; voltage-dependent anion channel 2 [KO:K15040]
22335  Vdac3; voltage-dependent anion channel 3 [KO:K15041]
11739  Slc25a4; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 4 [KO:K05863]
11740  Slc25a5; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 5 [KO:K05863]
73333  Slc25a31; solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31 [KO:K05863]
105675  Ppif; peptidylprolyl isomerase F (cyclophilin F) [KO:K09565] [EC:5.2.1.8]
11783  Apaf1; apoptotic peptidase activating factor 1 [KO:K02084]
13063  Cycs; cytochrome c, somatic [KO:K08738]
13067  Cyct; cytochrome c, testis [KO:K08738]
12371  Casp9; caspase 9 [KO:K04399] [EC:3.4.22.62]
12367  Casp3; caspase 3 [KO:K02187] [EC:3.4.22.56]
215999  Mcu; mitochondrial calcium uniporter [KO:K20858]
17716  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
17717  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
17718  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
17719  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
17720  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
17721  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
17722  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
17995  Ndufv1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2]
72900  Ndufv2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2]
78330  Ndufv3; NADH:ubiquinone oxidoreductase core subunit V3 [KO:K03944]
54405  Ndufa1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
17991  Ndufa2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
66091  Ndufa3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
17992  Ndufa4; Ndufa4, mitochondrial complex associated [KO:K03948]
407790  Ndufa4l2; Ndufa4, mitochondrial complex associated like 2 [KO:K03948]
68202  Ndufa5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
100502727  Gm19340; predicted gene, 19340 [KO:K03949]
67130  Ndufa6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
66416  Ndufa7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
68375  Ndufa8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
66108  Ndufa9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
67273  Ndufa10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
70316  Ndufab1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
102634451  Ndufab1-ps; NADH:ubiquinone oxidoreductase subunit AB1b [KO:K03955]
69875  Ndufa11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
66414  Ndufa12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
67184  Ndufa13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
102631912  Ndufb1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
68198  Ndufb2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
66495  Ndufb3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
68194  Ndufb4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
100042503  Ndufb4b; NADH:ubiquinone oxidoreductase subunit B4B [KO:K03960]
100041273  Ndufb4c; NADH:ubiquinone oxidoreductase subunit B4C [KO:K03960]
66046  Ndufb5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
230075  Ndufb6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
66916  Ndufb7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
67264  Ndufb8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
66218  Ndufb9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
68342  Ndufb10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
104130  Ndufb11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
227197  Ndufs1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2]
226646  Ndufs2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2]
68349  Ndufs3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2]
17993  Ndufs4; NADH:ubiquinone oxidoreductase core subunit S4 [KO:K03937]
595136  Ndufs5; NADH:ubiquinone oxidoreductase core subunit S5 [KO:K03938]
407785  Ndufs6; NADH:ubiquinone oxidoreductase core subunit S6 [KO:K03939]
623286  Ndufs6b; NADH:ubiquinone oxidoreductase subunit S6B [KO:K03939]
75406  Ndufs7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2]
225887  Ndufs8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2]
66377  Ndufc1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
68197  Ndufc2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
66945  Sdha; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234] [EC:1.3.5.1]
67680  Sdhb; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235] [EC:1.3.5.1]
66052  Sdhc; succinate dehydrogenase complex, subunit C, integral membrane protein [KO:K00236]
66925  Sdhd; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237]
66694  Uqcrfs1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:7.1.1.8]
17711  CYTB; cytochrome b [KO:K00412]
66445  Cyc1; cytochrome c-1 [KO:K00413]
22273  Uqcrc1; ubiquinol-cytochrome c reductase core protein 1 [KO:K00414]
67003  Uqcrc2; ubiquinol cytochrome c reductase core protein 2 [KO:K00415]
66576  Uqcrh; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
67530  Uqcrb; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
22272  Uqcrq; ubiquinol-cytochrome c reductase, complex III subunit VII [KO:K00418]
66152  Uqcr10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
102633050  Gm6293; predicted pseudogene 6293 [KO:K00419]
66594  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
17710  COX3; cytochrome c oxidase subunit III [KO:K02262]
17708  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
17709  COX2; cytochrome c oxidase subunit II [KO:K02261]
12857  Cox4i1; cytochrome c oxidase subunit 4I1 [KO:K02263]
84682  Cox4i2; cytochrome c oxidase subunit 4I2 [KO:K02263]
12858  Cox5a; cytochrome c oxidase subunit 5A [KO:K02264]
102633230  Gm11633; predicted gene 11633 [KO:K02264]
12859  Cox5b; cytochrome c oxidase subunit 5B [KO:K02265]
12861  Cox6a1; cytochrome c oxidase subunit 6A1 [KO:K02266]
12862  Cox6a2; cytochrome c oxidase subunit 6A2 [KO:K02266]
110323  Cox6b1; cytochrome c oxidase, subunit 6B1 [KO:K02267]
333182  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
12864  Cox6c; cytochrome c oxidase subunit 6C [KO:K02268]
12866  Cox7a2; cytochrome c oxidase subunit 7A2 [KO:K02270]
12865  Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270]
20463  Cox7a2l; cytochrome c oxidase subunit 7A2 like [KO:K02270]
66142  Cox7b; cytochrome c oxidase subunit 7B [KO:K02271]
78174  Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271]
12867  Cox7c; cytochrome c oxidase subunit 7C [KO:K02272]
12868  Cox8a; cytochrome c oxidase subunit 8A [KO:K02273]
12869  Cox8b; cytochrome c oxidase subunit 8B [KO:K02273]
75483  Cox8c; cytochrome c oxidase subunit 8C [KO:K02273]
11946  Atp5f1a; ATP synthase F1 subunit alpha [KO:K02132]
11947  Atp5f1b; ATP synthase F1 subunit beta [KO:K02133] [EC:7.1.2.2]
11949  Atp5f1c; ATP synthase F1 subunit gamma [KO:K02136]
66043  Atp5f1d; ATP synthase F1 subunit delta [KO:K02134]
67126  Atp5f1e; ATP synthase F1 subunit epsilon [KO:K02135]
17705  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
11950  Atp5pb; ATP synthase peripheral stalk-membrane subunit b [KO:K02127]
11951  Atp5mc1; ATP synthase membrane subunit c locus 1 [KO:K02128]
67942  Atp5mc2; ATP synthase membrane subunit c locus 2 [KO:K02128]
228033  Atp5mc3; ATP synthase membrane subunit c locus 3 [KO:K02128]
71679  Atp5pd; ATP synthase peripheral stalk subunit d [KO:K02138]
28080  Atp5po; ATP synthase peripheral stalk subunit OSCP [KO:K02137]
11957  Atp5pf; ATP synthase peripheral stalk subunit F6 [KO:K02131]
17706  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
26443  Psma6; proteasome subunit alpha 6 [KO:K02730] [EC:3.4.25.1]
19166  Psma2; proteasome subunit alpha 2 [KO:K02726] [EC:3.4.25.1]
26441  Psma4; proteasome subunit alpha 4 [KO:K02728] [EC:3.4.25.1]
26444  Psma7; proteasome subunit alpha 7 [KO:K02731] [EC:3.4.25.1]
73677  Psma8; proteasome subunit alpha 8 [KO:K02731] [EC:3.4.25.1]
26442  Psma5; proteasome subunit alpha 5 [KO:K02729] [EC:3.4.25.1]
26440  Psma1; proteasome subunit alpha 1 [KO:K02725] [EC:3.4.25.1]
19167  Psma3; proteasome subunit alpha 3 [KO:K02727] [EC:3.4.25.1]
19175  Psmb6; proteasome (prosome, macropain) subunit, beta type 6 [KO:K02738] [EC:3.4.25.1]
19177  Psmb7; proteasome (prosome, macropain) subunit, beta type 7 [KO:K02739] [EC:3.4.25.1]
26446  Psmb3; proteasome (prosome, macropain) subunit, beta type 3 [KO:K02735] [EC:3.4.25.1]
26445  Psmb2; proteasome (prosome, macropain) subunit, beta type 2 [KO:K02734] [EC:3.4.25.1]
19173  Psmb5; proteasome (prosome, macropain) subunit, beta type 5 [KO:K02737] [EC:3.4.25.1]
19170  Psmb1; proteasome (prosome, macropain) subunit, beta type 1 [KO:K02732] [EC:3.4.25.1]
19172  Psmb4; proteasome (prosome, macropain) subunit, beta type 4 [KO:K02736] [EC:3.4.25.1]
19181  Psmc2; proteasome (prosome, macropain) 26S subunit, ATPase 2 [KO:K03061]
19179  Psmc1; protease (prosome, macropain) 26S subunit, ATPase 1 [KO:K03062]
23996  Psmc4; proteasome (prosome, macropain) 26S subunit, ATPase, 4 [KO:K03063]
67089  Psmc6; proteasome (prosome, macropain) 26S subunit, ATPase, 6 [KO:K03064]
19182  Psmc3; proteasome (prosome, macropain) 26S subunit, ATPase 3 [KO:K03065]
19184  Psmc5; protease (prosome, macropain) 26S subunit, ATPase 5 [KO:K03066]
21762  Psmd2; proteasome (prosome, macropain) 26S subunit, non-ATPase, 2 [KO:K03028]
70247  Psmd1; proteasome (prosome, macropain) 26S subunit, non-ATPase, 1 [KO:K03032]
22123  Psmd3; proteasome (prosome, macropain) 26S subunit, non-ATPase, 3 [KO:K03033]
67151  Psmd9; proteasome (prosome, macropain) 26S subunit, non-ATPase, 9 [KO:K06693]
66997  Psmd12; proteasome (prosome, macropain) 26S subunit, non-ATPase, 12 [KO:K03035]
69077  Psmd11; proteasome (prosome, macropain) 26S subunit, non-ATPase, 11 [KO:K03036]
66413  Psmd6; proteasome (prosome, macropain) 26S subunit, non-ATPase, 6 [KO:K03037]
17463  Psmd7; proteasome (prosome, macropain) 26S subunit, non-ATPase, 7 [KO:K03038]
23997  Psmd13; proteasome (prosome, macropain) 26S subunit, non-ATPase, 13 [KO:K03039]
19185  Psmd4; proteasome (prosome, macropain) 26S subunit, non-ATPase, 4 [KO:K03029]
59029  Psmd14; proteasome (prosome, macropain) 26S subunit, non-ATPase, 14 [KO:K03030]
57296  Psmd8; proteasome (prosome, macropain) 26S subunit, non-ATPase, 8 [KO:K03031]
56436  Adrm1; adhesion regulating molecule 1 26S proteasome ubiquitin receptor [KO:K06691]
20422  Sem1; SEM1, 26S proteasome complex subunit [KO:K10881]
15139  Hc; hemolytic complement [KO:K03994]
12274  C6; complement component 6 [KO:K03995]
109828  C7; complement component 7 [KO:K03996]
230558  C8a; complement component 8, alpha polypeptide [KO:K03997]
110382  C8b; complement component 8, beta polypeptide [KO:K03998]
69379  C8g; complement component 8, gamma polypeptide [KO:K03999]
12279  C9; complement component 9 [KO:K04000]
12259  C1qa; complement component 1, q subcomponent, alpha polypeptide [KO:K03986]
12260  C1qb; complement component 1, q subcomponent, beta polypeptide [KO:K03987]
12262  C1qc; complement component 1, q subcomponent, C chain [KO:K03988]
15481  Hspa8; heat shock protein 8 [KO:K03283]
15482  Hspa1l; heat shock protein 1-like [KO:K03283]
193740  Hspa1a; heat shock protein 1A [KO:K03283]
15511  Hspa1b; heat shock protein 1B [KO:K03283]
18128  Notch1; notch 1 [KO:K02599]
12389  Cav1; caveolin 1, caveolae protein [KO:K06278]
12390  Cav2; caveolin 2 [KO:K12958]
12391  Cav3; caveolin 3 [KO:K12959]
18706  Pik3ca; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha [KO:K00922] [EC:2.7.1.153]
18707  Pik3cd; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit delta [KO:K00922] [EC:2.7.1.153]
74769  Pik3cb; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit beta [KO:K00922] [EC:2.7.1.153]
18709  Pik3r2; phosphoinositide-3-kinase regulatory subunit 2 [KO:K02649]
18708  Pik3r1; phosphoinositide-3-kinase regulatory subunit 1 [KO:K02649]
18710  Pik3r3; phosphoinositide-3-kinase regulatory subunit 3 [KO:K02649]
18753  Prkcd; protein kinase C, delta [KO:K06068] [EC:2.7.11.13]
13058  Cybb; cytochrome b-245, beta polypeptide [KO:K21421] [EC:1.-.-.-]
13057  Cyba; cytochrome b-245, alpha polypeptide [KO:K08009]
17972  Ncf4; neutrophil cytosolic factor 4 [KO:K08012]
17969  Ncf1; neutrophil cytosolic factor 1 [KO:K08011]
17970  Ncf2; neutrophil cytosolic factor 2 [KO:K08010]
19353  Rac1; Rac family small GTPase 1 [KO:K04392]
19354  Rac2; Rac family small GTPase 2 [KO:K07860]
26416  Mapk14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
19094  Mapk11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
29857  Mapk12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
26415  Mapk13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
26419  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
26420  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
26414  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
26413  Mapk1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
26417  Mapk3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
12912  Creb1; cAMP responsive element binding protein 1 [KO:K05870]
11909  Atf2; activating transcription factor 2 [KO:K04450]
12913  Creb3; cAMP responsive element binding protein 3 [KO:K09048]
26427  Creb3l1; cAMP responsive element binding protein 3-like 1 [KO:K09048]
208647  Creb3l2; cAMP responsive element binding protein 3-like 2 [KO:K09048]
208677  Creb3l3; cAMP responsive element binding protein 3-like 3 [KO:K09048]
78284  Creb3l4; cAMP responsive element binding protein 3-like 4 [KO:K09048]
231991  Creb5; cAMP responsive element binding protein 5 [KO:K09047]
12915  Atf6b; activating transcription factor 6 beta [KO:K09049]
13653  Egr1; early growth response 1 [KO:K09203]
20304  Ccl5; C-C motif chemokine ligand 5 [KO:K12499]
16175  Il1a; interleukin 1 alpha [KO:K04383]
16176  Il1b; interleukin 1 beta [KO:K04519]
16193  Il6; interleukin 6 [KO:K05405]
21926  Tnf; tumor necrosis factor [KO:K03156]
56637  Gsk3b; glycogen synthase kinase 3 beta [KO:K03083] [EC:2.7.11.26]
12995  Csnk2a1; casein kinase 2, alpha 1 polypeptide [KO:K03097] [EC:2.7.11.1]
13000  Csnk2a2; casein kinase 2, alpha prime polypeptide [KO:K03097] [EC:2.7.11.1]
13001  Csnk2b; casein kinase 2, beta polypeptide [KO:K03115]
16572  Kif5a; kinesin family member 5A [KO:K10396]
16573  Kif5b; kinesin family member 5B [KO:K10396]
16574  Kif5c; kinesin family member 5C [KO:K10396]
16593  Klc1; kinesin light chain 1 [KO:K10407]
16594  Klc2; kinesin light chain 2 [KO:K10407]
232943  Klc3; kinesin light chain 3 [KO:K10407]
74764  Klc4; kinesin light chain 4 [KO:K10407]
22145  Tuba4a; tubulin, alpha 4A [KO:K07374]
22144  Tuba3a; tubulin, alpha 3A [KO:K07374]
22142  Tuba1a; tubulin, alpha 1A [KO:K07374]
22146  Tuba1c; tubulin, alpha 1C [KO:K07374]
22147  Tuba3b; tubulin, alpha 3B [KO:K07374]
22143  Tuba1b; tubulin, alpha 1B [KO:K07374]
53857  Tuba8; tubulin, alpha 8 [KO:K07374]
238463  Tubal3; tubulin, alpha-like 3 [KO:K07374]
227613  Tubb4b; tubulin, beta 4B class IVB [KO:K07375]
22151  Tubb2a; tubulin, beta 2A class IIA [KO:K07375]
22152  Tubb3; tubulin, beta 3 class III [KO:K07375]
22153  Tubb4a; tubulin, beta 4A class IVA [KO:K07375]
67951  Tubb6; tubulin, beta 6 class V [KO:K07375]
22154  Tubb5; tubulin, beta 5 class I [KO:K07375]
545486  Tubb1; tubulin, beta 1 class VI [KO:K07375]
73710  Tubb2b; tubulin, beta 2B class IIB [KO:K07375]
Compound
C00027  Hydrogen peroxide
C00076  Calcium cation
C00575  3',5'-Cyclic AMP
C00704  Superoxide
C02140  Corticosterone
Reference
PMID:27055367
  Authors
Saa P, Harris DA, Cervenakova L
  Title
Mechanisms of prion-induced neurodegeneration.
  Journal
Expert Rev Mol Med 18:e5 (2016)
DOI:10.1017/erm.2016.8
Reference
PMID:25584786
  Authors
Goold R, McKinnon C, Tabrizi SJ
  Title
Prion degradation pathways: Potential for therapeutic intervention.
  Journal
Mol Cell Neurosci 66:12-20 (2015)
DOI:10.1016/j.mcn.2014.12.009
Reference
PMID:20889378
  Authors
Soto C, Satani N
  Title
The intricate mechanisms of neurodegeneration in prion diseases.
  Journal
Trends Mol Med 17:14-24 (2011)
DOI:10.1016/j.molmed.2010.09.001
Reference
PMID:27060771
  Authors
Mays CE, Soto C
  Title
The stress of prion disease.
  Journal
Brain Res 1648:553-560 (2016)
DOI:10.1016/j.brainres.2016.04.009
Reference
PMID:32565065
  Authors
Jones E, Mead S
  Title
Genetic risk factors for Creutzfeldt-Jakob disease.
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Neurobiol Dis 142:104973 (2020)
DOI:10.1016/j.nbd.2020.104973
Reference
PMID:18245809
  Authors
Kovacs GG, Budka H
  Title
Prion diseases: from protein to cell pathology.
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Am J Pathol 172:555-65 (2008)
DOI:10.2353/ajpath.2008.070442
Reference
PMID:15695097
  Authors
Campana V, Sarnataro D, Zurzolo C
  Title
The highways and byways of prion protein trafficking.
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Trends Cell Biol 15:102-11 (2005)
DOI:10.1016/j.tcb.2004.12.002
Reference
PMID:17051207
  Authors
Caughey B, Baron GS
  Title
Prions and their partners in crime.
  Journal
Nature 443:803-10 (2006)
DOI:10.1038/nature05294
Reference
PMID:11592845
  Authors
Chiesa R, Harris DA
  Title
Prion diseases: what is the neurotoxic molecule?
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Neurobiol Dis 8:743-63 (2001)
DOI:10.1006/nbdi.2001.0433
Reference
PMID:17085779
  Authors
Fasano C, Campana V, Zurzolo C
  Title
Prions: protein only or something more? Overview of potential prion cofactors.
  Journal
J Mol Neurosci 29:195-214 (2006)
DOI:10.1385/JMN:29:3:195
Reference
PMID:14705136
  Authors
Roucou X, Gains M, LeBlanc AC
  Title
Neuroprotective functions of prion protein.
  Journal
J Neurosci Res 75:153-61 (2004)
DOI:10.1002/jnr.10864
Reference
PMID:10398674
  Authors
Harris DA
  Title
Cellular biology of prion diseases.
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Clin Microbiol Rev 12:429-44 (1999)
Reference
PMID:15890824
  Authors
Novakofski J, Brewer MS, Mateus-Pinilla N, Killefer J, McCusker RH
  Title
Prion biology relevant to bovine spongiform encephalopathy.
  Journal
J Anim Sci 83:1455-76 (2005)
DOI:10.2527/2005.8361455x
Reference
PMID:27581199
  Authors
Peggion C, Bertoli A, Sorgato MC
  Title
Almost a century of prion protein(s): From pathology to physiology, and back to pathology.
  Journal
Biochem Biophys Res Commun 483:1148-1155 (2017)
DOI:10.1016/j.bbrc.2016.07.118
Reference
PMID:31618844
  Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
  Title
Prion Protein in Glioblastoma Multiforme.
  Journal
Int J Mol Sci 20:E5107 (2019)
DOI:10.3390/ijms20205107
Reference
PMID:31795242
  Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
  Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
  Journal
Int J Mol Sci 20:E6004 (2019)
DOI:10.3390/ijms20236004
Reference
PMID:30710214
  Authors
Meneghetti E, Gasperini L, Virgilio T, Moda F, Tagliavini F, Benetti F, Legname G
  Title
Prions Strongly Reduce NMDA Receptor S-Nitrosylation Levels at Pre-symptomatic and Terminal Stages of Prion Diseases.
  Journal
Mol Neurobiol 56:6035-6045 (2019)
DOI:10.1007/s12035-019-1505-6
Reference
PMID:23439666
  Authors
Song Z, Zhao D, Yang L
  Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
  Journal
Acta Biochim Biophys Sin (Shanghai) 45:452-64 (2013)
DOI:10.1093/abbs/gmt014
Reference
PMID:28643372
  Authors
Halliday M, Hughes D, Mallucci GR
  Title
Fine-tuning PERK signaling for neuroprotection.
  Journal
J Neurochem 142:812-826 (2017)
DOI:10.1111/jnc.14112
Reference
PMID:29194372
  Authors
Hughes D, Halliday M
  Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
  Journal
Pathogens 6:E63 (2017)
DOI:10.3390/pathogens6040063
Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
PMID:29476642
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
PMID:21722302
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
Reference
PMID:18420416
  Authors
Ferreiro E, Oliveira CR, Pereira CM
  Title
The release of calcium from the endoplasmic reticulum induced by amyloid-beta and prion peptides activates the mitochondrial apoptotic pathway.
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Neurobiol Dis 30:331-42 (2008)
DOI:10.1016/j.nbd.2008.02.003
Reference
PMID:16844381
  Authors
Ferreiro E, Resende R, Costa R, Oliveira CR, Pereira CM
  Title
An endoplasmic-reticulum-specific apoptotic pathway is involved in prion and amyloid-beta peptides neurotoxicity.
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Neurobiol Dis 23:669-78 (2006)
DOI:10.1016/j.nbd.2006.05.011
Reference
PMID:21980554
  Authors
Torres M, Encina G, Soto C, Hetz C
  Title
Abnormal calcium homeostasis and protein folding stress at the ER: A common factor in familial and infectious prion disorders.
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Commun Integr Biol 4:258-61 (2011)
DOI:10.4161/cib.4.3.15019
Reference
PMID:25766616
  Authors
Ciechanover A, Kwon YT
  Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
  Journal
Exp Mol Med 47:e147 (2015)
DOI:10.1038/emm.2014.117
Reference
PMID:17466621
  Authors
Kristiansen M, Deriziotis P, Dimcheff DE, Jackson GS, Ovaa H, Naumann H, Clarke AR, van Leeuwen FW, Menendez-Benito V, Dantuma NP, Portis JL, Collinge J, Tabrizi SJ
  Title
Disease-associated prion protein oligomers inhibit the 26S proteasome.
  Journal
Mol Cell 26:175-88 (2007)
DOI:10.1016/j.molcel.2007.04.001
Reference
PMID:10195903
  Authors
Wang HG, Pathan N, Ethell IM, Krajewski S, Yamaguchi Y, Shibasaki F, McKeon F, Bobo T, Franke TF, Reed JC.
  Title
Ca2+-induced apoptosis through calcineurin dephosphorylation of BAD.
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Science 284:339-43 (1999)
DOI:10.1126/science.284.5412.339
Reference
PMID:14532116
  Authors
Hetz C, Russelakis-Carneiro M, Maundrell K, Castilla J, Soto C
  Title
Caspase-12 and endoplasmic reticulum stress mediate neurotoxicity of pathological prion protein.
  Journal
EMBO J 22:5435-45 (2003)
DOI:10.1093/emboj/cdg537
Reference
PMID:31417367
  Authors
Zamponi E, Pigino GF
  Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
  Journal
Front Cell Neurosci 13:350 (2019)
DOI:10.3389/fncel.2019.00350
Reference
PMID:29261664
  Authors
Zamponi E, Buratti F, Cataldi G, Caicedo HH, Song Y, Jungbauer LM, LaDu MJ, Bisbal M, Lorenzo A, Ma J, Helguera PR, Morfini GA, Brady ST, Pigino GF
  Title
Prion protein inhibits fast axonal transport through a mechanism involving casein kinase 2.
  Journal
PLoS One 12:e0188340 (2017)
DOI:10.1371/journal.pone.0188340
Reference
PMID:21196165
  Authors
Schneider B, Pietri M, Pradines E, Loubet D, Launay JM, Kellermann O, Mouillet-Richard S
  Title
Understanding the neurospecificity of Prion protein signaling.
  Journal
Front Biosci (Landmark Ed) 16:169-86 (2011)
DOI:10.2741/3682
Reference
PMID:23479001
  Authors
Didonna A
  Title
Prion protein and its role in signal transduction.
  Journal
Cell Mol Biol Lett 18:209-30 (2013)
DOI:10.2478/s11658-013-0085-0
Reference
PMID:28507517
  Authors
Shah SZA, Zhao D, Hussain T, Yang L
  Title
The Role of Unfolded Protein Response and Mitogen-Activated Protein Kinase Signaling in Neurodegenerative Diseases with Special Focus on Prion Diseases.
  Journal
Front Aging Neurosci 9:120 (2017)
DOI:10.3389/fnagi.2017.00120
Reference
PMID:15528202
  Authors
Marella M, Gaggioli C, Batoz M, Deckert M, Tartare-Deckert S, Chabry J
  Title
Pathological prion protein exposure switches on neuronal mitogen-activated protein kinase pathway resulting in microglia recruitment.
  Journal
J Biol Chem 280:1529-34 (2005)
DOI:10.1074/jbc.M410966200
Reference
PMID:15640354
  Authors
Ishikura N, Clever JL, Bouzamondo-Bernstein E, Samayoa E, Prusiner SB, Huang EJ, DeArmond SJ
  Title
Notch-1 activation and dendritic atrophy in prion disease.
  Journal
Proc Natl Acad Sci U S A 102:886-91 (2005)
DOI:10.1073/pnas.0408612101
Reference
PMID:20205843
  Authors
Dearmond SJ, Bajsarowicz K
  Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
  Journal
Mol Neurodegener 5:6 (2010)
DOI:10.1186/1750-1326-5-6
Reference
PMID:29998397
  Authors
Hirsch TZ, Martin-Lanneree S, Reine F, Hernandez-Rapp J, Herzog L, Dron M, Privat N, Passet B, Halliez S, Villa-Diaz A, Lacroux C, Klein V, Haik S, Andreoletti O, Torres JM, Vilotte JL, Beringue V, Mouillet-Richard S
  Title
Epigenetic Control of the Notch and Eph Signaling Pathways by the Prion Protein: Implications for Prion Diseases.
  Journal
Mol Neurobiol 56:2159-2173 (2019)
DOI:10.1007/s12035-018-1193-7
Reference
PMID:28714865
  Authors
Aguzzi A, Zhu C
  Title
Microglia in prion diseases.
  Journal
J Clin Invest 127:3230-3239 (2017)
DOI:10.1172/JCI90605
Reference
PMID:15342004
  Authors
Mabbott NA
  Title
The complement system in prion diseases.
  Journal
Curr Opin Immunol 16:587-93 (2004)
DOI:10.1016/j.coi.2004.07.002
Reference
PMID:15611724
  Authors
Mallucci G, Collinge J
  Title
Rational targeting for prion therapeutics.
  Journal
Nat Rev Neurosci 6:23-34 (2005)
DOI:10.1038/nrn1584
Related
pathway
mmu00190  Oxidative phosphorylation
mmu03050  Proteasome
mmu04020  Calcium signaling pathway
mmu04140  Autophagy - animal
mmu04141  Protein processing in endoplasmic reticulum
mmu04142  Lysosome
mmu04144  Endocytosis
mmu04210  Apoptosis
mmu04330  Notch signaling pathway
mmu04610  Complement and coagulation cascades
mmu04668  TNF signaling pathway
KO pathway
ko05020   
LinkDB

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