KEGG   PATHWAY: hsa05022
Entry
hsa05022                    Pathway                                
Name
Pathways of neurodegeneration - multiple diseases - Homo sapiens (human)
Description
Neurodegeneration is generally defined as progressive, irreversible loss of neurons, which may affect either the peripheral or central nervous system (CNS). Neurodegenerative diseases (NDs) include highly debilitating illnesses, such as Alzheimer (AD), Parkinson disease (PD), amyotrophic lateral sclerosis, Huntington disease, spinocerebellar ataxias, and prion diseases (PrD). The hallmark event, which is thought to be at the root of these diseases, is the progressive accumulation of misfolded protein aggregates. Major basic processes include abnormal protein dynamics due to deficiency of the ubiquitin-proteosome-autophagy system, oxidative stress and free radical formation, endoplasmic reticulum stress, mitochondrial dysfunction and (secondary) disruptions of axonal transport.
Class
Human Diseases; Neurodegenerative disease
Pathway map
hsa05022  Pathways of neurodegeneration - multiple diseases
hsa05022

Drug
D04148  Fenfluramine hydrochloride (JAN/USAN)
D07945  Fenfluramine (INN)
D11293  Gosuranemab (USAN)
D11384  Hydromethylthionine mesylate (USAN)
D11427  Hydromethylthionine (USAN/INN)
D11533  Zagotenemab (USAN)
D11687  Semorinemab (USAN/INN)
D11811  Tofersen (JAN/USAN/INN)
D11840  Tilavonemab (USAN/INN)
D12228  Bepranemab (USAN/INN)
D12553  Nevadistinel (USAN)
D12636  Posdinemab (USAN/INN)
D12965  Indenebart (USAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
351  APP; amyloid beta precursor protein [KO:K04520]
4137  MAPT; microtubule associated protein tau [KO:K04380]
6622  SNCA; synuclein alpha [KO:K04528]
23435  TARDBP; TAR DNA binding protein [KO:K23600]
2521  FUS; FUS RNA binding protein [KO:K13098]
6647  SOD1; superoxide dismutase 1 [KO:K04565] [EC:1.15.1.1]
11273  ATXN2L; ataxin 2 like [KO:K23625]
6311  ATXN2; ataxin 2 [KO:K23625]
3064  HTT; huntingtin [KO:K04533]
342371  ATXN1L; ataxin 1 like [KO:K23616]
6310  ATXN1; ataxin 1 [KO:K23616]
4287  ATXN3; ataxin 3 [KO:K11863] [EC:3.4.22.-]
92552  ATXN3L; ataxin 3 like [KO:K11863] [EC:3.4.22.-]
5621  PRNP; prion protein (Kanno blood group) [KO:K05634]
1128  CHRM1; cholinergic receptor muscarinic 1 [KO:K04129]
1131  CHRM3; cholinergic receptor muscarinic 3 [KO:K04131]
1133  CHRM5; cholinergic receptor muscarinic 5 [KO:K04133]
6712  SPTBN2; spectrin beta, non-erythrocytic 2 [KO:K23932]
2911  GRM1; glutamate metabotropic receptor 1 [KO:K04603]
2915  GRM5; glutamate metabotropic receptor 5 [KO:K04604]
2776  GNAQ; G protein subunit alpha q [KO:K04634]
5071  PRKN; parkin RBR E3 ubiquitin protein ligase [KO:K04556] [EC:2.3.2.31]
23236  PLCB1; phospholipase C beta 1 [KO:K05858] [EC:3.1.4.11]
5330  PLCB2; phospholipase C beta 2 [KO:K05858] [EC:3.1.4.11]
5331  PLCB3; phospholipase C beta 3 [KO:K05858] [EC:3.1.4.11]
5332  PLCB4; phospholipase C beta 4 [KO:K05858] [EC:3.1.4.11]
5335  PLCG1; phospholipase C gamma 1 [KO:K01116] [EC:3.1.4.11]
5578  PRKCA; protein kinase C alpha [KO:K02677] [EC:2.7.11.13]
5579  PRKCB; protein kinase C beta [KO:K19662] [EC:2.7.11.13]
5582  PRKCG; protein kinase C gamma [KO:K19663] [EC:2.7.11.13]
7222  TRPC3; transient receptor potential cation channel subfamily C member 3 [KO:K04966]
10280  SIGMAR1; sigma non-opioid intracellular receptor 1 [KO:K20719]
3708  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
3709  ITPR2; inositol 1,4,5-trisphosphate receptor type 2 [KO:K04959]
3710  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
6261  RYR1; ryanodine receptor 1 [KO:K04961]
6262  RYR2; ryanodine receptor 2 [KO:K04962]
6263  RYR3; ryanodine receptor 3 [KO:K04963]
5173  PDYN; prodynorphin [KO:K15840]
1742  DLG4; discs large MAGUK scaffold protein 4 [KO:K11828]
2902  GRIN1; glutamate ionotropic receptor NMDA type subunit 1 [KO:K05208]
2903  GRIN2A; glutamate ionotropic receptor NMDA type subunit 2A [KO:K05209]
2904  GRIN2B; glutamate ionotropic receptor NMDA type subunit 2B [KO:K05210]
2905  GRIN2C; glutamate ionotropic receptor NMDA type subunit 2C [KO:K05211]
2906  GRIN2D; glutamate ionotropic receptor NMDA type subunit 2D [KO:K05212]
2890  GRIA1; glutamate ionotropic receptor AMPA type subunit 1 [KO:K05197]
2891  GRIA2; glutamate ionotropic receptor AMPA type subunit 2 [KO:K05198]
2892  GRIA3; glutamate ionotropic receptor AMPA type subunit 3 [KO:K05199]
2893  GRIA4; glutamate ionotropic receptor AMPA type subunit 4 [KO:K05200]
774  CACNA1B; calcium voltage-gated channel subunit alpha1 B [KO:K04849]
775  CACNA1C; calcium voltage-gated channel subunit alpha1 C [KO:K04850]
776  CACNA1D; calcium voltage-gated channel subunit alpha1 D [KO:K04851]
778  CACNA1F; calcium voltage-gated channel subunit alpha1 F [KO:K04853]
779  CACNA1S; calcium voltage-gated channel subunit alpha1 S [KO:K04857]
6804  STX1A; syntaxin 1A [KO:K04560]
1139  CHRNA7; cholinergic receptor nicotinic alpha 7 subunit [KO:K04809]
89832  CHRFAM7A; CHRNA7 (exons 5-10) and FAM7A (exons A-E) fusion [KO:K04809]
487  ATP2A1; ATPase sarcoplasmic/endoplasmic reticulum Ca2+ transporting 1 [KO:K05853] [EC:7.2.2.10]
489  ATP2A3; ATPase sarcoplasmic/endoplasmic reticulum Ca2+ transporting 3 [KO:K05853] [EC:7.2.2.10]
488  ATP2A2; ATPase sarcoplasmic/endoplasmic reticulum Ca2+ transporting 2 [KO:K05853] [EC:7.2.2.10]
5663  PSEN1; presenilin 1 [KO:K04505] [EC:3.4.23.-]
5664  PSEN2; presenilin 2 [KO:K04522] [EC:3.4.23.-]
79139  DERL1; derlin 1 [KO:K11519]
7415  VCP; valosin containing protein [KO:K13525]
7311  UBA52; ubiquitin A-52 residue ribosomal protein fusion product 1 [KO:K02927]
6233  RPS27A; ribosomal protein S27a [KO:K02977]
7314  UBB; ubiquitin B [KO:K04551]
7316  UBC; ubiquitin C [KO:K08770]
5687  PSMA6; proteasome 20S subunit alpha 6 [KO:K02730] [EC:3.4.25.1]
5683  PSMA2; proteasome 20S subunit alpha 2 [KO:K02726] [EC:3.4.25.1]
5685  PSMA4; proteasome 20S subunit alpha 4 [KO:K02728] [EC:3.4.25.1]
5688  PSMA7; proteasome 20S subunit alpha 7 [KO:K02731] [EC:3.4.25.1]
143471  PSMA8; proteasome 20S subunit alpha 8 [KO:K02731] [EC:3.4.25.1]
5686  PSMA5; proteasome 20S subunit alpha 5 [KO:K02729] [EC:3.4.25.1]
5682  PSMA1; proteasome 20S subunit alpha 1 [KO:K02725] [EC:3.4.25.1]
5684  PSMA3; proteasome 20S subunit alpha 3 [KO:K02727] [EC:3.4.25.1]
5694  PSMB6; proteasome 20S subunit beta 6 [KO:K02738] [EC:3.4.25.1]
5695  PSMB7; proteasome 20S subunit beta 7 [KO:K02739] [EC:3.4.25.1]
5691  PSMB3; proteasome 20S subunit beta 3 [KO:K02735] [EC:3.4.25.1]
5690  PSMB2; proteasome 20S subunit beta 2 [KO:K02734] [EC:3.4.25.1]
5693  PSMB5; proteasome 20S subunit beta 5 [KO:K02737] [EC:3.4.25.1]
5689  PSMB1; proteasome 20S subunit beta 1 [KO:K02732] [EC:3.4.25.1]
5692  PSMB4; proteasome 20S subunit beta 4 [KO:K02736] [EC:3.4.25.1]
5701  PSMC2; proteasome 26S subunit, ATPase 2 [KO:K03061]
5700  PSMC1; proteasome 26S subunit, ATPase 1 [KO:K03062]
5704  PSMC4; proteasome 26S subunit, ATPase 4 [KO:K03063]
5706  PSMC6; proteasome 26S subunit, ATPase 6 [KO:K03064]
5702  PSMC3; proteasome 26S subunit, ATPase 3 [KO:K03065]
5705  PSMC5; proteasome 26S subunit, ATPase 5 [KO:K03066]
5708  PSMD2; proteasome 26S subunit ubiquitin receptor, non-ATPase 2 [KO:K03028]
5707  PSMD1; proteasome 26S subunit, non-ATPase 1 [KO:K03032]
5709  PSMD3; proteasome 26S subunit, non-ATPase 3 [KO:K03033]
5715  PSMD9; proteasome 26S subunit, non-ATPase 9 [KO:K06693]
5718  PSMD12; proteasome 26S subunit, non-ATPase 12 [KO:K03035]
5717  PSMD11; proteasome 26S subunit, non-ATPase 11 [KO:K03036]
9861  PSMD6; proteasome 26S subunit, non-ATPase 6 [KO:K03037]
5713  PSMD7; proteasome 26S subunit, non-ATPase 7 [KO:K03038]
5719  PSMD13; proteasome 26S subunit, non-ATPase 13 [KO:K03039]
5710  PSMD4; proteasome 26S subunit ubiquitin receptor, non-ATPase 4 [KO:K03029]
10213  PSMD14; proteasome 26S subunit, non-ATPase 14 [KO:K03030]
5714  PSMD8; proteasome 26S subunit, non-ATPase 8 [KO:K03031]
11047  ADRM1; ADRM1 26S proteasome ubiquitin receptor [KO:K06691]
7979  SEM1; SEM1 26S proteasome subunit [KO:K10881]
7345  UCHL1; ubiquitin C-terminal hydrolase L1 [KO:K05611] [EC:3.4.19.12]
7317  UBA1; ubiquitin like modifier activating enzyme 1 [KO:K03178] [EC:6.2.1.45]
7318  UBA7; ubiquitin like modifier activating enzyme 7 [KO:K10698] [EC:6.2.1.45]
7332  UBE2L3; ubiquitin conjugating enzyme E2 L3 [KO:K04552] [EC:2.3.2.23]
9246  UBE2L6; ubiquitin conjugating enzyme E2 L6 [KO:K04553] [EC:2.3.2.23]
118424  UBE2J2; ubiquitin conjugating enzyme E2 J2 [KO:K04554] [EC:2.3.2.23]
51465  UBE2J1; ubiquitin conjugating enzyme E2 J1 [KO:K10578] [EC:2.3.2.23]
7327  UBE2G2; ubiquitin conjugating enzyme E2 G2 [KO:K04555] [EC:2.3.2.23]
7326  UBE2G1; ubiquitin conjugating enzyme E2 G1 [KO:K10575] [EC:2.3.2.23]
2861  GPR37; G protein-coupled receptor 37 [KO:K04243]
5413  SEPTIN5; septin 5 [KO:K04557]
9627  SNCAIP; synuclein alpha interacting protein [KO:K04558]
3309  HSPA5; heat shock protein family A (Hsp70) member 5 [KO:K09490] [EC:3.6.4.10]
9217  VAPB; VAMP associated protein B and C [KO:K10707]
22926  ATF6; activating transcription factor 6 [KO:K09054]
1649  DDIT3; DNA damage inducible transcript 3 [KO:K04452]
7494  XBP1; X-box binding protein 1 [KO:K09027]
2081  ERN1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
7186  TRAF2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
4217  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
5609  MAP2K7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
5599  MAPK8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
5602  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
5601  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
100506742  CASP12; caspase 12 (gene/pseudogene) [KO:K04741] [EC:3.4.22.-]
4294  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
9451  EIF2AK3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
1965  EIF2S1; eukaryotic translation initiation factor 2 subunit alpha [KO:K03237]
468  ATF4; activating transcription factor 4 [KO:K04374]
6531  SLC6A3; solute carrier family 6 member 3 [KO:K05036]
65018  PINK1; PTEN induced kinase 1 [KO:K05688] [EC:2.7.11.1]
4535  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
4536  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
4537  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
4538  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
4539  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
4540  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
4541  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
4723  NDUFV1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2]
4729  NDUFV2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2]
4731  NDUFV3; NADH:ubiquinone oxidoreductase subunit V3 [KO:K03944]
4694  NDUFA1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
4695  NDUFA2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
4696  NDUFA3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
4697  NDUFA4; NDUFA4 mitochondrial complex associated [KO:K03948]
56901  NDUFA4L2; NDUFA4 mitochondrial complex associated like 2 [KO:K03948]
107984365  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
4698  NDUFA5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
4700  NDUFA6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
4701  NDUFA7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
4702  NDUFA8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
4704  NDUFA9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
4705  NDUFA10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
4706  NDUFAB1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
126328  NDUFA11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
55967  NDUFA12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
51079  NDUFA13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
4707  NDUFB1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
4708  NDUFB2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
4709  NDUFB3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
4710  NDUFB4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
4711  NDUFB5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
4712  NDUFB6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
4713  NDUFB7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
4714  NDUFB8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
4715  NDUFB9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
4716  NDUFB10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
54539  NDUFB11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
4719  NDUFS1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2]
4720  NDUFS2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2]
4722  NDUFS3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2]
4724  NDUFS4; NADH:ubiquinone oxidoreductase subunit S4 [KO:K03937]
4725  NDUFS5; NADH:ubiquinone oxidoreductase subunit S5 [KO:K03938]
4726  NDUFS6; NADH:ubiquinone oxidoreductase subunit S6 [KO:K03939]
374291  NDUFS7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2]
4728  NDUFS8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2]
4717  NDUFC1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
4718  NDUFC2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
100532726  NDUFC2-KCTD14; NDUFC2-KCTD14 readthrough [KO:K03968]
6389  SDHA; succinate dehydrogenase complex flavoprotein subunit A [KO:K00234] [EC:1.3.5.1]
6390  SDHB; succinate dehydrogenase complex iron sulfur subunit B [KO:K00235] [EC:1.3.5.1]
6391  SDHC; succinate dehydrogenase complex subunit C [KO:K00236]
6392  SDHD; succinate dehydrogenase complex subunit D [KO:K00237]
7386  UQCRFS1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:7.1.1.8]
4519  CYTB; cytochrome b [KO:K00412]
1537  CYC1; cytochrome c1 [KO:K00413]
7384  UQCRC1; ubiquinol-cytochrome c reductase core protein 1 [KO:K00414]
7385  UQCRC2; ubiquinol-cytochrome c reductase core protein 2 [KO:K00415]
7388  UQCRH; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
440567  UQCRHL; ubiquinol-cytochrome c reductase hinge protein like [KO:K00416]
7381  UQCRB; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
27089  UQCRQ; ubiquinol-cytochrome c reductase complex III subunit VII [KO:K00418]
29796  UQCR10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
10975  UQCR11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
4514  COX3; cytochrome c oxidase subunit III [KO:K02262]
4512  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
4513  COX2; cytochrome c oxidase subunit II [KO:K02261]
84701  COX4I2; cytochrome c oxidase subunit 4I2 [KO:K02263]
1327  COX4I1; cytochrome c oxidase subunit 4I1 [KO:K02263]
9377  COX5A; cytochrome c oxidase subunit 5A [KO:K02264]
1329  COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
1337  COX6A1; cytochrome c oxidase subunit 6A1 [KO:K02266]
1339  COX6A2; cytochrome c oxidase subunit 6A2 [KO:K02266]
1340  COX6B1; cytochrome c oxidase subunit 6B1 [KO:K02267]
125965  COX6B2; cytochrome c oxidase subunit 6B2 [KO:K02267]
1345  COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
1346  COX7A1; cytochrome c oxidase subunit 7A1 [KO:K02270]
1347  COX7A2; cytochrome c oxidase subunit 7A2 [KO:K02270]
9167  COX7A2L; cytochrome c oxidase subunit 7A2 like [KO:K02270]
1349  COX7B; cytochrome c oxidase subunit 7B [KO:K02271]
170712  COX7B2; cytochrome c oxidase subunit 7B2 [KO:K02271]
1350  COX7C; cytochrome c oxidase subunit 7C [KO:K02272]
341947  COX8C; cytochrome c oxidase subunit 8C [KO:K02273]
1351  COX8A; cytochrome c oxidase subunit 8A [KO:K02273]
498  ATP5F1A; ATP synthase F1 subunit alpha [KO:K02132]
506  ATP5F1B; ATP synthase F1 subunit beta [KO:K02133] [EC:7.1.2.2]
509  ATP5F1C; ATP synthase F1 subunit gamma [KO:K02136]
513  ATP5F1D; ATP synthase F1 subunit delta [KO:K02134]
514  ATP5F1E; ATP synthase F1 subunit epsilon [KO:K02135]
4508  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
515  ATP5PB; ATP synthase peripheral stalk-membrane subunit b [KO:K02127]
516  ATP5MC1; ATP synthase membrane subunit c locus 1 [KO:K02128]
517  ATP5MC2; ATP synthase membrane subunit c locus 2 [KO:K02128]
518  ATP5MC3; ATP synthase membrane subunit c locus 3 [KO:K02128]
10476  ATP5PD; ATP synthase peripheral stalk subunit d [KO:K02138]
539  ATP5PO; ATP synthase peripheral stalk subunit OSCP [KO:K02137]
522  ATP5PF; ATP synthase peripheral stalk subunit F6 [KO:K02131]
4509  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
596  BCL2; BCL2 apoptosis regulator [KO:K02161]
7416  VDAC1; voltage dependent anion channel 1 [KO:K05862]
10452  TOMM40; translocase of outer mitochondrial membrane 40 [KO:K11518]
84134  TOMM40L; translocase of outer mitochondrial membrane 40 like [KO:K11518]
90550  MCU; mitochondrial calcium uniporter [KO:K20858]
810  CALML3; calmodulin like 3 [KO:K02183]
805  CALM2; calmodulin 2 [KO:K02183]
808  CALM3; calmodulin 3 [KO:K02183]
801  CALM1; calmodulin 1 [KO:K02183]
163688  CALML6; calmodulin like 6 [KO:K02183]
51806  CALML5; calmodulin like 5 [KO:K02183]
91860  CALML4; calmodulin like 4 [KO:K02183]
5530  PPP3CA; protein phosphatase 3 catalytic subunit alpha [KO:K04348] [EC:3.1.3.16]
5532  PPP3CB; protein phosphatase 3 catalytic subunit beta [KO:K04348] [EC:3.1.3.16]
5533  PPP3CC; protein phosphatase 3 catalytic subunit gamma [KO:K04348] [EC:3.1.3.16]
5534  PPP3R1; protein phosphatase 3 regulatory subunit B, alpha [KO:K06268]
5535  PPP3R2; protein phosphatase 3 regulatory subunit B, beta [KO:K06268]
572  BAD; BCL2 associated agonist of cell death [KO:K02158]
356  FASLG; Fas ligand [KO:K04389]
355  FAS; Fas cell surface death receptor [KO:K04390]
8772  FADD; Fas associated via death domain [KO:K02373]
841  CASP8; caspase 8 [KO:K04398] [EC:3.4.22.61]
637  BID; BH3 interacting domain death agonist [KO:K04726]
581  BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
578  BAK1; BCL2 antagonist/killer 1 [KO:K14021]
120892  LRRK2; leucine rich repeat kinase 2 [KO:K08844] [EC:2.7.11.1]
27429  HTRA2; HtrA serine peptidase 2 [KO:K08669] [EC:3.4.21.108]
10131  TRAP1; TNF receptor associated protein 1 [KO:K09488]
7417  VDAC2; voltage dependent anion channel 2 [KO:K15040]
7419  VDAC3; voltage dependent anion channel 3 [KO:K15041]
291  SLC25A4; solute carrier family 25 member 4 [KO:K05863]
292  SLC25A5; solute carrier family 25 member 5 [KO:K05863]
293  SLC25A6; solute carrier family 25 member 6 [KO:K05863]
83447  SLC25A31; solute carrier family 25 member 31 [KO:K05863]
10105  PPIF; peptidylprolyl isomerase F [KO:K09565] [EC:5.2.1.8]
54205  CYCS; cytochrome c, somatic [KO:K08738]
317  APAF1; apoptotic peptidase activating factor 1 [KO:K02084]
842  CASP9; caspase 9 [KO:K04399] [EC:3.4.22.62]
836  CASP3; caspase 3 [KO:K02187] [EC:3.4.22.56]
3092  HIP1; huntingtin interacting protein 1 [KO:K04559]
55081  IFT57; intraflagellar transport 57 [KO:K04638]
3028  HSD17B10; hydroxysteroid 17-beta dehydrogenase 10 [KO:K08683] [EC:1.1.1.35 1.1.1.178]
5481  PPID; peptidylprolyl isomerase D [KO:K05864] [EC:5.2.1.8]
11315  PARK7; Parkinsonism associated deglycase [KO:K05687] [EC:3.5.1.124]
598  BCL2L1; BCL2 like 1 [KO:K04570]
55669  MFN1; mitofusin 1 [KO:K21356] [EC:3.6.5.-]
9927  MFN2; mitofusin 2 [KO:K06030] [EC:3.6.5.-]
10133  OPTN; optineurin [KO:K19946]
8878  SQSTM1; sequestosome 1 [KO:K14381]
440738  MAP1LC3C; microtubule associated protein 1 light chain 3 gamma [KO:K10435]
81631  MAP1LC3B; microtubule associated protein 1 light chain 3 beta [KO:K10435]
84557  MAP1LC3A; microtubule associated protein 1 light chain 3 alpha [KO:K10435]
643246  MAP1LC3B2; microtubule associated protein 1 light chain 3 beta 2 [KO:K10435]
392288  MAP1LC3BP1; microtubule associated protein 1 light chain 3 beta pseudogene 1 [KO:K10435]
29110  TBK1; TANK binding kinase 1 [KO:K05410] [EC:2.7.11.10]
10010  TANK; TRAF family member associated NFKB activator [KO:K12650]
55255  WDR41; WD repeat domain 41 [KO:K23610]
140775  SMCR8; SMCR8-C9orf72 complex subunit [KO:K23611]
203228  C9orf72; C9orf72-SMCR8 complex subunit [KO:K23609]
4218  RAB8A; RAB8A, member RAS oncogene family [KO:K07901]
116442  RAB39B; RAB39B, member RAS oncogene family [KO:K07925]
5861  RAB1A; RAB1A, member RAS oncogene family [KO:K07874]
2475  MTOR; mechanistic target of rapamycin kinase [KO:K07203] [EC:2.7.11.1]
8408  ULK1; unc-51 like autophagy activating kinase 1 [KO:K21357] [EC:2.7.11.1]
9706  ULK2; unc-51 like autophagy activating kinase 2 [KO:K08269] [EC:2.7.11.1]
60673  ATG101; autophagy related 101 [KO:K19730]
9776  ATG13; autophagy related 13 [KO:K08331]
9821  RB1CC1; RB1 inducible coiled-coil 1 [KO:K17589]
55626  AMBRA1; autophagy and beclin 1 regulator 1 [KO:K17985]
8678  BECN1; beclin 1 [KO:K08334]
441925  BECN2; beclin 2 [KO:K08334]
30849  PIK3R4; phosphoinositide-3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
22863  ATG14; autophagy related 14 [KO:K17889]
29982  NRBF2; nuclear receptor binding factor 2 [KO:K21246]
5289  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
23130  ATG2A; autophagy related 2A [KO:K17906]
55102  ATG2B; autophagy related 2B [KO:K17906]
26100  WIPI2; WD repeat domain, phosphoinositide interacting 2 [KO:K17908]
55062  WIPI1; WD repeat domain, phosphoinositide interacting 1 [KO:K17908]
53349  ZFYVE1; zinc finger FYVE-type containing 1 [KO:K17603]
57679  ALS2; alsin Rho guanine nucleotide exchange factor ALS2 [KO:K04575]
5868  RAB5A; RAB5A, member RAS oncogene family [KO:K07887]
5879  RAC1; Rac family small GTPase 1 [KO:K04392]
25978  CHMP2B; charged multivesicular body protein 2B [KO:K12192]
9896  FIG4; FIG4 phosphoinositide 5-phosphatase [KO:K22913] [EC:3.1.3.-]
80208  SPG11; SPG11 vesicle trafficking associated, spatacsin [KO:K19026]
7124  TNF; tumor necrosis factor [KO:K03156]
7132  TNFRSF1A; TNF receptor superfamily member 1A [KO:K03158]
7133  TNFRSF1B; TNF receptor superfamily member 1B [KO:K05141]
1616  DAXX; death domain associated protein [KO:K02308]
5606  MAP2K3; mitogen-activated protein kinase kinase 3 [KO:K04432] [EC:2.7.12.2]
5608  MAP2K6; mitogen-activated protein kinase kinase 6 [KO:K04433] [EC:2.7.12.2]
5600  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
6300  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
5603  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
1432  MAPK14; mitogen-activated protein kinase 14 [KO:K04441] [EC:2.7.11.24]
4842  NOS1; nitric oxide synthase 1 [KO:K13240] [EC:1.14.13.39]
9973  CCS; copper chaperone for superoxide dismutase [KO:K04569]
257202  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
2882  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
2877  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
2878  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
2876  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
2880  GPX5; glutathione peroxidase 5 [KO:K00432] [EC:1.11.1.9]
493869  GPX8; glutathione peroxidase 8 (putative) [KO:K00432] [EC:1.11.1.9]
847  CAT; catalase [KO:K03781] [EC:1.11.1.6]
5630  PRPH; peripherin [KO:K07607]
4747  NEFL; neurofilament light chain [KO:K04572]
4741  NEFM; neurofilament medium chain [KO:K04573]
4744  NEFH; neurofilament heavy chain [KO:K04574]
3798  KIF5A; kinesin family member 5A [KO:K10396] [EC:5.6.1.3]
3799  KIF5B; kinesin family member 5B [KO:K10396] [EC:5.6.1.3]
3800  KIF5C; kinesin family member 5C [KO:K10396] [EC:5.6.1.3]
147700  KLC3; kinesin light chain 3 [KO:K10407]
3831  KLC1; kinesin light chain 1 [KO:K10407]
64837  KLC2; kinesin light chain 2 [KO:K10407]
89953  KLC4; kinesin light chain 4 [KO:K10407]
10376  TUBA1B; tubulin alpha 1b [KO:K07374]
7277  TUBA4A; tubulin alpha 4a [KO:K07374]
7278  TUBA3C; tubulin alpha 3c [KO:K07374]
7846  TUBA1A; tubulin alpha 1a [KO:K07374]
84790  TUBA1C; tubulin alpha 1c [KO:K07374]
51807  TUBA8; tubulin alpha 8 [KO:K07374]
112714  TUBA3E; tubulin alpha 3e [KO:K07374]
113457  TUBA3D; tubulin alpha 3d [KO:K07374]
79861  TUBAL3; tubulin alpha like 3 [KO:K07374]
84617  TUBB6; tubulin beta 6 class V [KO:K07375]
203068  TUBB; tubulin beta class I [KO:K07375]
81027  TUBB1; tubulin beta 1 class VI [KO:K07375]
7280  TUBB2A; tubulin beta 2A class IIa [KO:K07375]
10381  TUBB3; tubulin beta 3 class III [KO:K07375]
10382  TUBB4A; tubulin beta 4A class IVa [KO:K07375]
347688  TUBB8; tubulin beta 8 class VIII [KO:K07375]
347733  TUBB2B; tubulin beta 2B class IIb [KO:K07375]
10383  TUBB4B; tubulin beta 4B class IVb [KO:K07375]
124908013  tubulin beta 8B [KO:K07375]
124908015  tubulin beta 8B [KO:K07375]
124908041  tubulin beta 8B [KO:K07375]
260334  TUBB8B; tubulin beta 8B [KO:K07375]
2932  GSK3B; glycogen synthase kinase 3 beta [KO:K03083] [EC:2.7.11.26]
1457  CSNK2A1; casein kinase 2 alpha 1 [KO:K03097] [EC:2.7.11.1]
1459  CSNK2A2; casein kinase 2 alpha 2 [KO:K03097] [EC:2.7.11.1]
283106  CSNK2A3; casein kinase 2 alpha 3 [KO:K03097] [EC:2.7.11.1]
1460  CSNK2B; casein kinase 2 beta [KO:K03115]
627  BDNF; brain derived neurotrophic factor [KO:K04355]
1639  DCTN1; dynactin subunit 1 [KO:K04648]
10540  DCTN2; dynactin subunit 2 [KO:K10424]
11258  DCTN3; dynactin subunit 3 [KO:K10425]
51164  DCTN4; dynactin subunit 4 [KO:K10426]
84516  DCTN5; dynactin subunit 5 [KO:K10427]
10671  DCTN6; dynactin subunit 6 [KO:K10428]
10121  ACTR1A; actin related protein 1A [KO:K16575]
10120  ACTR1B; actin related protein 1B [KO:K16575]
55860  ACTR10; actin related protein 10 [KO:K16576]
9001  HAP1; huntingtin associated protein 1 [KO:K04647]
25981  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
55567  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
146754  DNAH2; dynein axonemal heavy chain 2 [KO:K10408]
56171  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
1767  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
1768  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
1770  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
8701  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
201625  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
127602  DNAH14; dynein axonemal heavy chain 14 [KO:K10408]
1769  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
196385  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
8632  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
7802  DNALI1; dynein axonemal light intermediate chain 1 [KO:K10410]
27019  DNAI1; dynein axonemal intermediate chain 1 [KO:K10409]
64446  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
83544  DNAL1; dynein axonemal light chain 1 [KO:K10411]
10126  DNAL4; dynein axonemal light chain 4 [KO:K10412]
815  CAMK2A; calcium/calmodulin dependent protein kinase II alpha [KO:K04515] [EC:2.7.11.17]
817  CAMK2D; calcium/calmodulin dependent protein kinase II delta [KO:K04515] [EC:2.7.11.17]
816  CAMK2B; calcium/calmodulin dependent protein kinase II beta [KO:K04515] [EC:2.7.11.17]
818  CAMK2G; calcium/calmodulin dependent protein kinase II gamma [KO:K04515] [EC:2.7.11.17]
823  CAPN1; calpain 1 [KO:K01367] [EC:3.4.22.52]
824  CAPN2; calpain 2 [KO:K03853] [EC:3.4.22.53]
8851  CDK5R1; cyclin dependent kinase 5 regulatory subunit 1 [KO:K11716]
1020  CDK5; cyclin dependent kinase 5 [KO:K02090] [EC:2.7.11.22]
840  CASP7; caspase 7 [KO:K04397] [EC:3.4.22.60]
22943  DKK1; dickkopf WNT signaling pathway inhibitor 1 [KO:K02165]
27123  DKK2; dickkopf WNT signaling pathway inhibitor 2 [KO:K02165]
27121  DKK4; dickkopf WNT signaling pathway inhibitor 4 [KO:K02165]
7471  WNT1; Wnt family member 1 [KO:K03209]
7472  WNT2; Wnt family member 2 [KO:K00182]
7482  WNT2B; Wnt family member 2B [KO:K00182]
7473  WNT3; Wnt family member 3 [KO:K00312]
89780  WNT3A; Wnt family member 3A [KO:K00312]
54361  WNT4; Wnt family member 4 [KO:K00408]
7474  WNT5A; Wnt family member 5A [KO:K00444]
81029  WNT5B; Wnt family member 5B [KO:K00444]
7475  WNT6; Wnt family member 6 [KO:K00445]
7476  WNT7A; Wnt family member 7A [KO:K00572]
7477  WNT7B; Wnt family member 7B [KO:K00572]
7478  WNT8A; Wnt family member 8A [KO:K00714]
7479  WNT8B; Wnt family member 8B [KO:K00714]
7483  WNT9A; Wnt family member 9A [KO:K01064]
7484  WNT9B; Wnt family member 9B [KO:K01064]
7480  WNT10B; Wnt family member 10B [KO:K01357]
80326  WNT10A; Wnt family member 10A [KO:K01357]
7481  WNT11; Wnt family member 11 [KO:K01384]
51384  WNT16; Wnt family member 16 [KO:K01558]
8321  FZD1; frizzled class receptor 1 [KO:K02432]
8324  FZD7; frizzled class receptor 7 [KO:K02432]
2535  FZD2; frizzled class receptor 2 [KO:K02235]
7976  FZD3; frizzled class receptor 3 [KO:K02329]
8322  FZD4; frizzled class receptor 4 [KO:K02354]
7855  FZD5; frizzled class receptor 5 [KO:K02375]
8325  FZD8; frizzled class receptor 8 [KO:K02375]
8323  FZD6; frizzled class receptor 6 [KO:K02376]
11211  FZD10; frizzled class receptor 10 [KO:K02842]
8326  FZD9; frizzled class receptor 9 [KO:K02842]
4041  LRP5; LDL receptor related protein 5 [KO:K03068]
4040  LRP6; LDL receptor related protein 6 [KO:K03068]
1454  CSNK1E; casein kinase 1 epsilon [KO:K08960] [EC:2.7.11.1]
102800317  TPTEP2-CSNK1E; TPTEP2-CSNK1E readthrough [KO:K08960] [EC:2.7.11.1]
1857  DVL3; dishevelled segment polarity protein 3 [KO:K02353]
1856  DVL2; dishevelled segment polarity protein 2 [KO:K02353]
1855  DVL1; dishevelled segment polarity protein 1 [KO:K02353]
10023  FRAT1; FRAT regulator of WNT signaling pathway 1 [KO:K03069]
23401  FRAT2; FRAT regulator of WNT signaling pathway 2 [KO:K03096]
8312  AXIN1; axin 1 [KO:K02157]
8313  AXIN2; axin 2 [KO:K04385]
324  APC; APC regulator of WNT signaling pathway [KO:K02085]
10297  APC2; APC regulator of WNT signaling pathway 2 [KO:K02085]
1499  CTNNB1; catenin beta 1 [KO:K02105]
122011  CSNK1A1L; casein kinase 1 alpha 1 like [KO:K08957] [EC:2.7.11.1]
1452  CSNK1A1; casein kinase 1 alpha 1 [KO:K08957] [EC:2.7.11.1]
177  AGER; advanced glycosylation end-product specific receptor [KO:K19722]
3265  HRAS; HRas proto-oncogene, GTPase [KO:K02833]
3845  KRAS; KRAS proto-oncogene, GTPase [KO:K07827]
4893  NRAS; NRAS proto-oncogene, GTPase [KO:K07828]
369  ARAF; A-Raf proto-oncogene, serine/threonine kinase [KO:K08845] [EC:2.7.11.1]
673  BRAF; B-Raf proto-oncogene, serine/threonine kinase [KO:K04365] [EC:2.7.11.1]
5894  RAF1; Raf-1 proto-oncogene, serine/threonine kinase [KO:K04366] [EC:2.7.11.1]
5604  MAP2K1; mitogen-activated protein kinase kinase 1 [KO:K04368] [EC:2.7.12.2]
5605  MAP2K2; mitogen-activated protein kinase kinase 2 [KO:K04369] [EC:2.7.12.2]
5594  MAPK1; mitogen-activated protein kinase 1 [KO:K04371] [EC:2.7.11.24]
5595  MAPK3; mitogen-activated protein kinase 3 [KO:K04371] [EC:2.7.11.24]
27035  NOX1; NADPH oxidase 1 [KO:K08008]
1536  CYBB; cytochrome b-245 beta chain [KO:K21421] [EC:1.-.-.-]
50507  NOX4; NADPH oxidase 4 [KO:K21423] [EC:1.6.3.-]
5970  RELA; RELA proto-oncogene, NF-kB subunit [KO:K04735]
4790  NFKB1; nuclear factor kappa B subunit 1 [KO:K02580]
4843  NOS2; nitric oxide synthase 2 [KO:K13241] [EC:1.14.13.39]
5743  PTGS2; prostaglandin-endoperoxide synthase 2 [KO:K11987] [EC:1.14.99.1]
3569  IL6; interleukin 6 [KO:K05405]
3552  IL1A; interleukin 1 alpha [KO:K04383]
3553  IL1B; interleukin 1 beta [KO:K04519]
1435  CSF1; colony stimulating factor 1 [KO:K05453]
Compound
C00001  H2O
C00002  ATP
C00008  ADP
C00009  Orthophosphate
C00012  Peptide
C00013  Diphosphate
C00017  Protein
C00020  AMP
C00025  L-Glutamate
C00027  Hydrogen peroxide
C00062  L-Arginine
C00070  Copper
C00076  Calcium cation
C00165  Diacylglycerol
C00533  Nitric oxide
C00704  Superoxide
C01245  D-myo-Inositol 1,4,5-trisphosphate
C01330  Sodium cation
C01996  Acetylcholine
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
C04599  1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine
C07593  Rotenone
C11310  1-Methyl-4-phenylpyridinium
C15231  Maneb
C16514  Amyloid beta protein 40
C16515  Amyloid beta protein 42
C16844  Hydroxyl radical
C16845  Peroxynitrite
C21011  [Protein]-N(epsilon)-(carboxymethyl)lysine
C21012  [Protein]-N(epsilon)-(carboxyethyl)lysine
C21013  [Protein]-pyrraline
C21014  [Protein]-pentosidine
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Converging pathways in neurodegeneration, from genetics to mechanisms.
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Nat Neurosci 21:1300-1309 (2018)
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Basic mechanisms of neurodegeneration: a critical update.
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Front Mol Neurosci 7:70 (2014)
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Neurodegener Dis 14:161-75 (2014)
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Autophagy in Neurodegenerative Diseases: A Hunter for Aggregates.
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Int J Mol Sci 21:E3369 (2020)
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Mol Brain 12:100 (2019)
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Autophagy in Age-Associated Neurodegeneration.
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Cells 7:E37 (2018)
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Targeting autophagy in neurodegenerative diseases.
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Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities.
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Neuron 93:1015-1034 (2017)
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Reference
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Chu CT
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Mechanisms of selective autophagy and mitophagy: Implications for neurodegenerative diseases.
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Neurobiol Dis 122:23-34 (2019)
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Kim I, Xu W, Reed JC
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Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities.
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Nat Rev Drug Discov 7:1013-30 (2008)
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J Cell Mol Med 15:2025-39 (2011)
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Fine-tuning PERK signaling for neuroprotection.
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J Neurochem 142:812-826 (2017)
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Int J Mol Sci 21:E2108 (2020)
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Calcium signaling and neurodegenerative diseases.
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Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment.
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CNS Neurosci Ther 23:5-22 (2017)
DOI:10.1111/cns.12655
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Morfini GA, Burns M, Binder LI, Kanaan NM, LaPointe N, Bosco DA, Brown RH Jr, Brown H, Tiwari A, Hayward L, Edgar J, Nave KA, Garberrn J, Atagi Y, Song Y, Pigino G, Brady ST
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Axonal transport defects in neurodegenerative diseases.
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J Neurosci 29:12776-86 (2009)
DOI:10.1523/JNEUROSCI.3463-09.2009
Reference
  Authors
Gibbs KL, Greensmith L, Schiavo G
  Title
Regulation of Axonal Transport by Protein Kinases.
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Trends Biochem Sci 40:597-610 (2015)
DOI:10.1016/j.tibs.2015.08.003
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Millecamps S, Julien JP
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Axonal transport deficits and neurodegenerative diseases.
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Nat Rev Neurosci 14:161-76 (2013)
DOI:10.1038/nrn3380
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Sleigh JN, Rossor AM, Fellows AD, Tosolini AP, Schiavo G
  Title
Axonal transport and neurological disease.
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Nat Rev Neurol 15:691-703 (2019)
DOI:10.1038/s41582-019-0257-2
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Perlson E, Maday S, Fu MM, Moughamian AJ, Holzbaur EL
  Title
Retrograde axonal transport: pathways to cell death?
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Trends Neurosci 33:335-44 (2010)
DOI:10.1016/j.tins.2010.03.006
Related
pathway
hsa00190  Oxidative phosphorylation
hsa03050  Proteasome
hsa04020  Calcium signaling pathway
hsa04120  Ubiquitin mediated proteolysis
hsa04137  Mitophagy - animal
hsa04140  Autophagy - animal
hsa04141  Protein processing in endoplasmic reticulum
hsa04142  Lysosome
hsa04210  Apoptosis
hsa04310  Wnt signaling pathway
hsa04724  Glutamatergic synapse
hsa04933  AGE-RAGE signaling pathway in diabetic complications
KO pathway
ko05022   
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