Entry
Name
Prion disease - Rattus norvegicus (rat)
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
BRITE hierarchy
Pathway map
Disease
Organism
Rattus norvegicus (rat) [GN:
rno ]
Gene
100188937 Cox7c; cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272 ]
100360357 Cox7cl1; cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272 ]
100361833 Atp5mc2l1; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
100361934 Ndufb4l4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960 ]
100362331 Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
100911483 Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
100912357 Ndufb1; nADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957 ]
103690609 Ndufb3l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959 ]
103691107 Ndufb4l5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960 ]
103691697 Ndufb3l2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959 ]
103694107 Ndufb3l3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959 ]
114493 Cacna1f; voltage-dependent L-type calcium channel subunit alpha-1F [KO:K04853 ]
114630 Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial precursor [KO:K02128 ]
116550 Atp5f1c; ATP synthase subunit gamma, mitochondrial [KO:K02136 ]
117262 Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063 ]
117263 Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062 ]
157074 Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial precursor [KO:K00234 ] [EC:1.3.5.1 ]
161475 Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693 ]
170796 Grin3b; glutamate receptor ionotropic, NMDA 3B precursor [KO:K05214 ]
170911 Pik3ca; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit alpha isoform [KO:K00922 ] [EC:2.7.1.153 ]
171082 Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial precursor [KO:K02128 ]
171335 Cox8a; cytochrome c oxidase subunit 8A, mitochondrial precursor [KO:K02273 ]
171375 Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial precursor [KO:K02127 ]
171378 Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform [KO:K04348 ] [EC:3.1.3.16 ]
191573 Grin3a; glutamate receptor ionotropic, NMDA 3A isoform 1 precursor [KO:K05213 ]
192241 Atp5po; ATP synthase subunit O, mitochondrial precursor [KO:K02137 ]
24237 C6; complement component C6 precursor [KO:K03995 ]
24239 Cacna1c; voltage-dependent L-type calcium channel subunit alpha-1C [KO:K04850 ]
24330 Egr1; early growth response protein 1 [KO:K09203 ]
24408 Grin1; glutamate receptor ionotropic, NMDA 1 isoform 1a precursor [KO:K05208 ]
24409 Grin2a; glutamate receptor ionotropic, NMDA 2A precursor [KO:K05209 ]
24410 Grin2b; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210 ]
24411 Grin2c; glutamate receptor ionotropic, NMDA 2C [KO:K05211 ]
24412 Grin2d; glutamate receptor ionotropic, NMDA 2D precursor [KO:K05212 ]
24468 Hspa8; heat shock cognate 71 kDa protein [KO:K03283 ]
24586 Ncam1; neural cell adhesion molecule 1 isoform 5 precursor [KO:K06491 ]
245958 Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135 ]
245965 Atp5f1d; ATP synthase subunit delta, mitochondrial precursor [KO:K02134 ]
24675 Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform [KO:K04348 ] [EC:3.1.3.16 ]
24963 Hspa1l; heat shock 70 kDa protein 1-like [KO:K03283 ]
25250 Cox8b; cytochrome c oxidase subunit 8B, mitochondrial precursor [KO:K02273 ]
25262 Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform 3 [KO:K04958 ]
25278 Cox6a2; cytochrome c oxidase subunit 6A2, mitochondrial precursor [KO:K02266 ]
25282 Cox6a1; cytochrome c oxidase subunit 6A1, mitochondrial precursor [KO:K02266 ]
252934 Cox5a; cytochrome c oxidase subunit 5A, mitochondrial precursor [KO:K02264 ]
25488 Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
25496 Notch1; neurogenic locus notch homolog protein 1 precursor [KO:K02599 ]
25513 Pik3r1; phosphatidylinositol 3-kinase regulatory subunit alpha [KO:K02649 ]
25581 Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061 ]
25679 Itpr3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960 ]
257648 Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform 1 [KO:K04849 ]
26198 COX2; cytochrome c oxidase subunit II [KO:K02261 ]
26204 COX3; cytochrome c oxidase subunit III [KO:K02262 ]
282819 Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial precursor [KO:K09565 ] [EC:5.2.1.8 ]
287670 Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033 ]
287772 Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035 ]
287984 Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028 ]
288088 Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
288280 Ncam2; neural cell adhesion molecule 2 precursor [KO:K06491 ]
289217 Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236 ]
289218 Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial precursor [KO:K03935 ] [EC:7.1.1.2 ]
289924 Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037 ]
289990 Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064 ]
291660 Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
292766 Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031 ]
293130 Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968 ]
293448 Uqcrc2; cytochrome b-c1 complex subunit 2, mitochondrial precursor [KO:K00415 ]
293453 Ndufab1; acyl carrier protein, mitochondrial [KO:K03955 ]
293652 Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941 ] [EC:7.1.1.2 ]
293655 Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942 ] [EC:7.1.1.2 ]
293991 Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964 ]
29445 Cox4i1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial precursor [KO:K02263 ]
294560 Mcu; calcium uniporter protein, mitochondrial precursor [KO:K20858 ]
29467 Ddit3; DNA damage-inducible transcript 3 protein [KO:K04452 ]
294964 Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961 ]
29507 Cox7a2; cytochrome c oxidase subunit 7A2, mitochondrial precursor [KO:K02270 ]
295923 Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936 ] [EC:7.1.1.2 ]
296545 C8g; complement component C8 gamma chain isoform 1 precursor [KO:K03999 ]
296658 Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
29677 Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065 ]
29687 C1qb; complement C1q subcomponent subunit B precursor [KO:K03987 ]
29702 Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 precursor [KO:K08860 ] [EC:2.7.11.1 ]
29716 Cacna1d; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851 ]
29741 Pik3r2; phosphatidylinositol 3-kinase regulatory subunit beta [KO:K02649 ]
29754 Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial precursor [KO:K02128 ]
297990 Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962 ]
298288 C8a; complement component C8 alpha chain precursor [KO:K03997 ]
298400 Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048 ]
298566 C1qa; complement C1q subcomponent subunit A precursor [KO:K03986 ]
298596 Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235 ] [EC:1.3.5.1 ]
298762 Cox7a2l; cytochrome c oxidase subunit 7A2 like isoform 1 [KO:K02270 ]
299310 Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
299643 Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951 ]
299739 Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
299954 Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965 ]
301011 Uqcrc1; cytochrome b-c1 complex subunit 1, mitochondrial precursor [KO:K00414 ]
301123 Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
301427 Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
301458 Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial precursor [KO:K03934 ] [EC:7.1.1.2 ]
303353 Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036 ]
303393 Cox7b; cytochrome c oxidase subunit 7B, mitochondrial precursor [KO:K02271 ]
307821 Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038 ]
310616 Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform 1 [KO:K09048 ]
311078 Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030 ]
313421 C8b; complement component C8 beta chain precursor [KO:K03998 ]
314638 Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048 ]
315167 Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
316632 Ndufa10l1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10-like [KO:K03954 ]
360229 Cox8c; cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273 ]
361385 Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
362165 Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048 ]
362339 Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048 ]
362344 Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958 ]
362440 Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
362588 Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
362634 C1qc; complement C1q subcomponent subunit C precursor [KO:K03988 ]
362837 Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940 ] [EC:7.1.1.2 ]
363061 Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237 ]
363441 Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
363875 Rac1; ras-related C3 botulinum toxin substrate 1 [KO:K04392 ]
365388 Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039 ]
366448 Uqcrh; cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416 ]
366508 Pik3cd; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit delta isoform [KO:K00922 ] [EC:2.7.1.153 ]
366614 Atp5mc1l2; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128 ]
366957 Rac2; ras-related C3 botulinum toxin substrate 2 [KO:K07860 ]
406169 Atf6b; cyclic AMP-dependent transcription factor ATF-6 beta [KO:K09049 ]
498885 Slc25a6; solute carrier family 25 member 6 [KO:K05863 ]
499131 Ndufb4l3; similar to NADH:ubiquinone oxidoreductase B15 subunit [KO:K03960 ]
499512 Atp5mc2l2; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
499529 Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial precursor [KO:K03937 ]
500131 Creb5; cyclic AMP-responsive element-binding protein 5 [KO:K09047 ]
501886 Ndufb4l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960 ]
54318 Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
54322 Cox6c; cytochrome c oxidase subunit 6C-2 [KO:K02268 ]
60664 Pik3r3; phosphatidylinositol 3-kinase regulatory subunit gamma [KO:K02649 ]
641434 Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138 ]
64539 Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform 2 [KO:K03944 ]
64639 Bad; bcl2-associated agonist of cell death [KO:K02158 ]
65138 Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691 ]
65262 Atp5f1a; ATP synthase subunit alpha, mitochondrial precursor [KO:K02132 ]
66021 Cybb; cytochrome b-245 beta chain [KO:K21421 ] [EC:1.-.-.-]
678759 Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial precursor [KO:K03954 ]
679739 Ndufs6; NADH:ubiquinone oxidoreductase subunit S6-like 1 [KO:K03939 ]
680121 Haus8l2; heat shock cognate 71 kDa protein-like [KO:K03283 ]
680532 Sem1; 26S proteasome complex subunit SEM1 [KO:K10881 ]
681024 Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948 ]
681418 Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
682930 Cacna1s; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857 ]
685322 Uqcr10; ubiquinol-cytochrome c reductase complex 7.2kDa protein [KO:K00419 ]
685596 Uqcrbl1; cytochrome b-c1 complex subunit 7-like [KO:K00417 ]
688022 Atp5pdl1; ATP synthase subunit d, mitochondrial-like [KO:K02138 ]
688386 Cox7a2l2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270 ]
689938 Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
689961 Atp5mc1l1; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128 ]
690848 Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420 ]
691001 Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
78963 Apaf1; apoptotic protease-activating factor 1 [KO:K02084 ]
79255 Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
81646 Creb1; cyclic AMP-responsive element-binding protein 1 isoform A [KO:K05870 ]
81647 Atf2; cyclic AMP-dependent transcription factor ATF-2 [KO:K04450 ]
81650 Csnk2b; casein kinase II subunit beta [KO:K03115 ]
81678 Itpr2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959 ]
81728 Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial precursor [KO:K03943 ] [EC:7.1.1.2 ]
81780 Ccl5; C-C motif chemokine 5 precursor [KO:K12499 ]
81827 Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066 ]
83499 Psmd4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029 ]
83529 Vdac1; voltage-dependent anion-selective channel protein 1 [KO:K05862 ]
83531 Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040 ]
83532 Vdac3; voltage-dependent anion-selective channel protein 3 [KO:K15041 ]
83806 Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032 ]
84683 Cox4i2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform 2 precursor [KO:K02263 ]
85243 Pik3cb; phosphatidylinositol 4,5-bisphosphate 3-kinase catalytic subunit beta isoform [KO:K00922 ] [EC:2.7.1.153 ]
94194 Cox5b; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265 ]
94271 Atp5pf; ATP synthase-coupling factor 6, mitochondrial precursor [KO:K02131 ]
Compound
Reference
Authors
Saa P, Harris DA, Cervenakova L
Title
Mechanisms of prion-induced neurodegeneration.
Journal
Reference
Authors
Goold R, McKinnon C, Tabrizi SJ
Title
Prion degradation pathways: Potential for therapeutic intervention.
Journal
Reference
Authors
Soto C, Satani N
Title
The intricate mechanisms of neurodegeneration in prion diseases.
Journal
Reference
Authors
Mays CE, Soto C
Title
The stress of prion disease.
Journal
Reference
Authors
Jones E, Mead S
Title
Genetic risk factors for Creutzfeldt-Jakob disease.
Journal
Reference
Authors
Kovacs GG, Budka H
Title
Prion diseases: from protein to cell pathology.
Journal
Reference
Authors
Campana V, Sarnataro D, Zurzolo C
Title
The highways and byways of prion protein trafficking.
Journal
Reference
Authors
Caughey B, Baron GS
Title
Prions and their partners in crime.
Journal
Reference
Authors
Chiesa R, Harris DA
Title
Prion diseases: what is the neurotoxic molecule?
Journal
Reference
Authors
Fasano C, Campana V, Zurzolo C
Title
Prions: protein only or something more? Overview of potential prion cofactors.
Journal
Reference
Authors
Roucou X, Gains M, LeBlanc AC
Title
Neuroprotective functions of prion protein.
Journal
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
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
Reference
Authors
Ryskalin L, Busceti CL, Biagioni F, Limanaqi F, Familiari P, Frati A, Fornai F
Title
Prion Protein in Glioblastoma Multiforme.
Journal
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
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
Reference
Authors
Song Z, Zhao D, Yang L
Title
Molecular mechanisms of neurodegeneration mediated by dysfunctional subcellular organelles in transmissible spongiform encephalopathies.
Journal
Reference
Authors
Halliday M, Hughes D, Mallucci GR
Title
Fine-tuning PERK signaling for neuroprotection.
Journal
Reference
Authors
Hughes D, Halliday M
Title
What Is Our Current Understanding of PrP(Sc)-Associated Neurotoxicity and Its Molecular Underpinnings?
Journal
Reference
Authors
Hetz C, Mollereau B
Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
Journal
Reference
Authors
Hughes D, Mallucci GR
Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
Journal
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
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
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
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
Reference
Authors
Ciechanover A, Kwon YT
Title
Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies.
Journal
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
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
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
Reference
Authors
Zamponi E, Pigino GF
Title
Protein Misfolding, Signaling Abnormalities and Altered Fast Axonal Transport: Implications for Alzheimer and Prion Diseases.
Journal
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
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
Reference
Authors
Didonna A
Title
Prion protein and its role in signal transduction.
Journal
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
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
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
Reference
Authors
Dearmond SJ, Bajsarowicz K
Title
PrPSc accumulation in neuronal plasma membranes links Notch-1 activation to dendritic degeneration in prion diseases.
Journal
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
Reference
Authors
Aguzzi A, Zhu C
Title
Microglia in prion diseases.
Journal
Reference
Authors
Mabbott NA
Title
The complement system in prion diseases.
Journal
Reference
Authors
Mallucci G, Collinge J
Title
Rational targeting for prion therapeutics.
Journal
Related pathway
rno04141 Protein processing in endoplasmic reticulum
rno04610 Complement and coagulation cascades
KO pathway