KEGG   PATHWAY: ko05020
Entry
ko05020                     Pathway                                
Name
Prion disease
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
ko05020  Prion disease
ko05020

Disease
H00061  Prion disease
Orthology
K00234  SDHA, SDH1; succinate dehydrogenase (ubiquinone) flavoprotein subunit [EC:1.3.5.1]
K00235  SDHB, SDH2; succinate dehydrogenase (ubiquinone) iron-sulfur subunit [EC:1.3.5.1]
K00236  SDHC, SDH3; succinate dehydrogenase (ubiquinone) cytochrome b560 subunit
K00237  SDHD, SDH4; succinate dehydrogenase (ubiquinone) membrane anchor subunit
K00411  UQCRFS1, RIP1, petA; ubiquinol-cytochrome c reductase iron-sulfur subunit [EC:7.1.1.8]
K00412  CYTB, petB; ubiquinol-cytochrome c reductase cytochrome b subunit
K00413  CYC1, CYT1, petC; ubiquinol-cytochrome c reductase cytochrome c1 subunit
K00414  QCR1, UQCRC1; ubiquinol-cytochrome c reductase core subunit 1
K00415  QCR2, UQCRC2; ubiquinol-cytochrome c reductase core subunit 2
K00416  QCR6, UQCRH; ubiquinol-cytochrome c reductase subunit 6
K00417  QCR7, UQCRB; ubiquinol-cytochrome c reductase subunit 7
K00418  QCR8, UQCRQ; ubiquinol-cytochrome c reductase subunit 8
K00419  QCR9, UCRC; ubiquinol-cytochrome c reductase subunit 9
K00420  QCR10, UQCR; ubiquinol-cytochrome c reductase subunit 10
K00922  PIK3CA_B_D; phosphatidylinositol-4,5-bisphosphate 3-kinase catalytic subunit alpha/beta/delta [EC:2.7.1.153]
K02084  APAF1; apoptotic protease-activating factor
K02125  ATPeF08, MTATP8, ATP8; F-type H+-transporting ATPase subunit 8
K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
K02127  ATPeF0B, ATP5F1, ATP4; F-type H+-transporting ATPase subunit b
K02128  ATPeF0C, ATP5G, ATP9; F-type H+-transporting ATPase subunit c
K02131  ATPeF0F6, ATP5J; F-type H+-transporting ATPase subunit 6
K02132  ATPeF1A, ATP5A1, ATP1; F-type H+-transporting ATPase subunit alpha
K02133  ATPeF1B, ATP5B, ATP2; F-type H+-transporting ATPase subunit beta [EC:7.1.2.2]
K02134  ATPeF1D, ATP5D, ATP16; F-type H+-transporting ATPase subunit delta
K02135  ATPeF1E, ATP5E, ATP15; F-type H+-transporting ATPase subunit epsilon
K02136  ATPeF1G, ATP5C1, ATP3; F-type H+-transporting ATPase subunit gamma
K02137  ATPeF0O, ATP5O, ATP5; F-type H+-transporting ATPase subunit O
K02138  ATPeF0D, ATP5H, ATP7; F-type H+-transporting ATPase subunit d
K02158  BAD; Bcl-2-antagonist of cell death
K02159  BAX; apoptosis regulator BAX
K02187  CASP3; caspase 3 [EC:3.4.22.56]
K02256  COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
K02261  COX2; cytochrome c oxidase subunit 2
K02262  COX3; cytochrome c oxidase subunit 3
K02263  COX4; cytochrome c oxidase subunit 4
K02264  COX5A; cytochrome c oxidase subunit 5a
K02265  COX5B; cytochrome c oxidase subunit 5b
K02266  COX6A; cytochrome c oxidase subunit 6a
K02267  COX6B; cytochrome c oxidase subunit 6b
K02268  COX6C; cytochrome c oxidase subunit 6c
K02270  COX7A; cytochrome c oxidase subunit 7a
K02271  COX7B; cytochrome c oxidase subunit 7b
K02272  COX7C; cytochrome c oxidase subunit 7c
K02273  COX8; cytochrome c oxidase subunit 8
K02599  NOTCH1; Notch 1
K02649  PIK3R1_2_3; phosphoinositide-3-kinase regulatory subunit alpha/beta/delta
K02725  PSMA1; 20S proteasome subunit alpha 6 [EC:3.4.25.1]
K02726  PSMA2; 20S proteasome subunit alpha 2 [EC:3.4.25.1]
K02727  PSMA3; 20S proteasome subunit alpha 7 [EC:3.4.25.1]
K02728  PSMA4; 20S proteasome subunit alpha 3 [EC:3.4.25.1]
K02729  PSMA5; 20S proteasome subunit alpha 5 [EC:3.4.25.1]
K02730  PSMA6; 20S proteasome subunit alpha 1 [EC:3.4.25.1]
K02731  PSMA7; 20S proteasome subunit alpha 4 [EC:3.4.25.1]
K02732  PSMB1; 20S proteasome subunit beta 6 [EC:3.4.25.1]
K02734  PSMB2; 20S proteasome subunit beta 4 [EC:3.4.25.1]
K02735  PSMB3; 20S proteasome subunit beta 3 [EC:3.4.25.1]
K02736  PSMB4; 20S proteasome subunit beta 7 [EC:3.4.25.1]
K02737  PSMB5; 20S proteasome subunit beta 5 [EC:3.4.25.1]
K02738  PSMB6; 20S proteasome subunit beta 1 [EC:3.4.25.1]
K02739  PSMB7; 20S proteasome subunit beta 2 [EC:3.4.25.1]
K03028  PSMD2, RPN1; 26S proteasome regulatory subunit N1
K03029  PSMD4, RPN10; 26S proteasome regulatory subunit N10
K03030  PSMD14, RPN11, POH1; 26S proteasome regulatory subunit N11
K03031  PSMD8, RPN12; 26S proteasome regulatory subunit N12
K03032  PSMD1, RPN2; 26S proteasome regulatory subunit N2
K03033  PSMD3, RPN3; 26S proteasome regulatory subunit N3
K03035  PSMD12, RPN5; 26S proteasome regulatory subunit N5
K03036  PSMD11, RPN6; 26S proteasome regulatory subunit N6
K03037  PSMD6, RPN7; 26S proteasome regulatory subunit N7
K03038  PSMD7, RPN8; 26S proteasome regulatory subunit N8
K03039  PSMD13, RPN9; 26S proteasome regulatory subunit N9
K03061  PSMC2, RPT1; 26S proteasome regulatory subunit T1
K03062  PSMC1, RPT2; 26S proteasome regulatory subunit T2
K03063  PSMC4, RPT3; 26S proteasome regulatory subunit T3
K03064  PSMC6, RPT4; 26S proteasome regulatory subunit T4
K03065  PSMC3, RPT5; 26S proteasome regulatory subunit T5
K03066  PSMC5, RPT6; 26S proteasome regulatory subunit T6
K03083  GSK3B; glycogen synthase kinase 3 beta [EC:2.7.11.26]
K03097  CSNK2A; casein kinase II subunit alpha [EC:2.7.11.1]
K03115  CSNK2B; casein kinase II subunit beta
K03156  TNF, TNFA; tumor necrosis factor superfamily, member 2
K03237  EIF2S1; translation initiation factor 2 subunit 1
K03283  HSPA1_6_8; heat shock 70kDa protein 1/6/8
K03878  ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
K03879  ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
K03880  ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
K03881  ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
K03882  ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
K03883  ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
K03884  ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
K03934  NDUFS1; NADH dehydrogenase (ubiquinone) Fe-S protein 1 [EC:7.1.1.2]
K03935  NDUFS2; NADH dehydrogenase (ubiquinone) Fe-S protein 2 [EC:7.1.1.2]
K03936  NDUFS3; NADH dehydrogenase (ubiquinone) Fe-S protein 3 [EC:7.1.1.2]
K03937  NDUFS4; NADH dehydrogenase (ubiquinone) Fe-S protein 4
K03938  NDUFS5; NADH dehydrogenase (ubiquinone) Fe-S protein 5
K03939  NDUFS6; NADH dehydrogenase (ubiquinone) Fe-S protein 6
K03940  NDUFS7; NADH dehydrogenase (ubiquinone) Fe-S protein 7 [EC:7.1.1.2]
K03941  NDUFS8; NADH dehydrogenase (ubiquinone) Fe-S protein 8 [EC:7.1.1.2]
K03942  NDUFV1; NADH dehydrogenase (ubiquinone) flavoprotein 1 [EC:7.1.1.2]
K03943  NDUFV2; NADH dehydrogenase (ubiquinone) flavoprotein 2 [EC:7.1.1.2]
K03944  NDUFV3; NADH dehydrogenase (ubiquinone) flavoprotein 3
K03945  NDUFA1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 1
K03946  NDUFA2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 2
K03947  NDUFA3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 3
K03948  NDUFA4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4
K03949  NDUFA5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 5
K03950  NDUFA6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 6
K03951  NDUFA7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 7
K03952  NDUFA8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 8
K03953  NDUFA9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 9
K03954  NDUFA10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 10
K03955  NDUFAB1; NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1, acyl-carrier protein
K03956  NDUFA11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 11
K03957  NDUFB1; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 1
K03958  NDUFB2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 2
K03959  NDUFB3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 3
K03960  NDUFB4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 4
K03961  NDUFB5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 5
K03962  NDUFB6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6
K03963  NDUFB7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7
K03964  NDUFB8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8
K03965  NDUFB9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9
K03966  NDUFB10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10
K03967  NDUFC1; NADH dehydrogenase (ubiquinone) 1 subunit C1
K03968  NDUFC2; NADH dehydrogenase (ubiquinone) 1 subunit C2
K03986  C1QA; complement C1q subcomponent subunit A
K03987  C1QB; complement C1q subcomponent subunit B
K03988  C1QG; complement C1q subcomponent subunit C
K03994  C5; complement component 5
K03995  C6; complement component 6
K03996  C7; complement component 7
K03997  C8A; complement component 8 subunit alpha
K03998  C8B; complement component 8 subunit beta
K03999  C8G; complement component 8 subunit gamma
K04000  C9; complement component 9
K04345  PKA; protein kinase A [EC:2.7.11.11]
K04348  PPP3C, CNA; serine/threonine-protein phosphatase 2B catalytic subunit [EC:3.1.3.16]
K04371  ERK, MAPK1_3; mitogen-activated protein kinase 1/3 [EC:2.7.11.24]
K04374  ATF4, CREB2; cyclic AMP-dependent transcription factor ATF-4
K04383  IL1A; interleukin 1 alpha
K04392  RAC1; Ras-related C3 botulinum toxin substrate 1
K04399  CASP9; caspase 9 [EC:3.4.22.62]
K04440  JNK; mitogen-activated protein kinase 8/9/10 (c-Jun N-terminal kinase) [EC:2.7.11.24]
K04441  P38; p38 MAP kinase [EC:2.7.11.24]
K04450  ATF2, CREBP1; cyclic AMP-dependent transcription factor ATF-2
K04452  DDIT3, GADD153; DNA damage-inducible transcript 3
K04519  IL1B; interleukin 1 beta
K04565  SOD1; superoxide dismutase, Cu-Zn family [EC:1.15.1.1]
K04741  CASP12; caspase 12 [EC:3.4.22.-]
K04849  CACNA1B, CAV2.2; voltage-dependent calcium channel N type alpha-1B
K04850  CACNA1C, CAV1.2; voltage-dependent calcium channel L type alpha-1C
K04851  CACNA1D, CAV1.3; voltage-dependent calcium channel L type alpha-1D
K04853  CACNA1F, CAV1.4; voltage-dependent calcium channel L type alpha-1F
K04857  CACNA1S, CAV1.1; voltage-dependent calcium channel L type alpha-1S
K04958  ITPR1; inositol 1,4,5-triphosphate receptor type 1
K04959  ITPR2; inositol 1,4,5-triphosphate receptor type 2
K04960  ITPR3; inositol 1,4,5-triphosphate receptor type 3
K04961  RYR1; ryanodine receptor 1
K04962  RYR2; ryanodine receptor 2
K04963  RYR3; ryanodine receptor 3
K05208  GRIN1; glutamate receptor ionotropic, NMDA 1
K05209  GRIN2A; glutamate receptor ionotropic, NMDA 2A
K05210  GRIN2B; glutamate receptor ionotropic, NMDA 2B
K05211  GRIN2C; glutamate receptor ionotropic, NMDA 2C
K05212  GRIN2D; glutamate receptor ionotropic, NMDA 2D
K05213  GRIN3A; glutamate receptor ionotropic, NMDA 3A
K05214  GRIN3B; glutamate receptor ionotropic, NMDA 3B
K05405  IL6; interleukin 6
K05634  PRNP, PrP, CD230; prion protein
K05635  LAMC1; laminin, gamma 1
K05703  FYN; tyrosine-protein kinase Fyn [EC:2.7.10.2]
K05862  VDAC1; voltage-dependent anion channel protein 1
K05863  SLC25A4S, ANT; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
K05870  CREB1; cyclic AMP-responsive element-binding protein 1
K06068  PRKCD; novel protein kinase C delta type [EC:2.7.11.13]
K06268  PPP3R, CNB; serine/threonine-protein phosphatase 2B regulatory subunit
K06278  CAV1; caveolin 1
K06491  NCAM, CD56; neural cell adhesion molecule
K06691  RPN13; 26S proteasome regulatory subunit N13
K06693  PSMD9, RPN4; 26S proteasome regulatory subunit N4
K07374  TUBA; tubulin alpha
K07375  TUBB; tubulin beta
K07860  RAC2; Ras-related C3 botulinum toxin substrate 2
K08009  CYBA, P22PHOX; cytochrome b-245, alpha polypeptide
K08010  NCF2, P67PHOX; neutrophil cytosolic factor 2
K08011  NCF1, P47PHOX; neutrophil cytosolic factor 1
K08012  NCF4, P40PHOX; neutrophil cytosolic factor 4
K08738  CYC; cytochrome c
K08860  EIF2AK3, PERK; eukaryotic translation initiation factor 2-alpha kinase 3 [EC:2.7.11.1]
K09047  CREB5, CREBPA; cyclic AMP-responsive element-binding protein 5
K09048  CREB3; cyclic AMP-responsive element-binding protein 3
K09049  ATF6B, CREBL1; cyclic AMP-dependent transcription factor ATF-6 beta
K09203  EGR1; early growth response protein 1
K09490  HSPA5, BIP; endoplasmic reticulum chaperone BiP [EC:3.6.4.10]
K09553  STIP1; stress-induced-phosphoprotein 1
K09565  PPIF; peptidyl-prolyl isomerase F (cyclophilin D) [EC:5.2.1.8]
K10396  KIF5; kinesin family member 5 (plus-end-directed kinesin ATPase) [EC:5.6.1.3]
K10407  KLC; kinesin light chain
K10881  SHFM1, DSS1, RPN15; 26 proteasome complex subunit DSS1
K11351  NDUFB11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11
K11352  NDUFA12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 12
K11353  NDUFA13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13
K12499  CCL5; C-C motif chemokine 5
K12958  CAV2; caveolin 2
K12959  CAV3; caveolin 3
K15040  VDAC2; voltage-dependent anion channel protein 2
K15041  VDAC3; voltage-dependent anion channel protein 3
K20858  MCU; calcium uniporter protein, mitochondrial
K21421  NOX2, GP91, CYBB; NADPH oxidase 2 [EC:1.-.-.-]
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.
  Journal
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.
  Journal
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.
  Journal
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?
  Journal
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.
  Journal
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.
  Journal
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.
  Journal
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.
  Journal
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.
  Journal
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)
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Related
pathway
ko00190  Oxidative phosphorylation
ko03050  Proteasome
ko04020  Calcium signaling pathway
ko04140  Autophagy - animal
ko04141  Protein processing in endoplasmic reticulum
ko04142  Lysosome
ko04144  Endocytosis
ko04210  Apoptosis
ko04330  Notch signaling pathway
ko04610  Complement and coagulation cascades
ko04668  TNF signaling pathway

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