KEGG   PATHWAY: cge05014
Entry
cge05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Cricetulus griseus (Chinese hamster)
Description
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by a progressive degeneration of motor neurons in the brain and spinal cord. In 90% of patients, ALS is sporadic, with no clear genetic linkage. On the other hand, the remaining 10% of cases show familial inheritance, with mutations in SOD1, TDP43(TARDBP), FUS, or C9orf72 genes being the most frequent causes. In spite of such difference, familial ALS and sporadic ALS have similarities in their pathological features. Proposed disease mechanisms contributing to motor neuron degeneration in ALS are: impaired proteostasis, aberrant RNA processing, mitochondrial disfunction and oxidative stress, microglia activation, and axonal dysfunction.
Class
Human Diseases; Neurodegenerative disease
Pathway map
cge05014  Amyotrophic lateral sclerosis
cge05014

Organism
Cricetulus griseus (Chinese hamster) [GN:cge]
Gene
100682525  Tp53; cellular tumor antigen p53 [KO:K04451]
100759171  caspase-1 isoform X1 [KO:K01370] [EC:3.4.22.36]
100754606  Bid; BH3-interacting domain death agonist [KO:K04726]
100774873  Bcl2; apoptosis regulator Bcl-2 isoform X3 [KO:K02161]
100689371  Bcl2l1; bcl-2-like protein 1 [KO:K04570]
100689032  Bax; apoptosis regulator BAX [KO:K02159]
100760426  Bad; bcl2-associated agonist of cell death isoform X2 [KO:K02158]
100758500  apoptotic protease-activating factor 1 isoform X4 [KO:K02084]
107977792  cytochrome c-like [KO:K08738]
103160806  cytochrome c-like [KO:K08738]
100757098  cytochrome c, somatic [KO:K08738]
100764460  cytochrome c, testis-specific [KO:K08738]
100773626  cytochrome c, somatic [KO:K08738]
100754043  cytochrome c, somatic [KO:K08738]
100768478  cytochrome c [KO:K08738]
103164112  cytochrome c-like [KO:K08738]
100751857  cytochrome c, somatic-like [KO:K08738]
103160479  caspase-9-like [KO:K04399] [EC:3.4.22.62]
100689370  caspase-9 [KO:K04399] [EC:3.4.22.62]
113833881  caspase-9-like [KO:K04399] [EC:3.4.22.62]
100689061  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100765091  Sod1; superoxide dismutase [Cu-Zn] isoform X2 [KO:K04565] [EC:1.15.1.1]
3979183  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
3979184  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
3979190  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
3979179  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
3979178  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
3979180  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
3979181  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100757245  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100757242  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X3 [KO:K03943] [EC:7.1.1.2]
100761076  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
100689328  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100754444  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
100764988  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
118238483  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
100766611  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100755007  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100760870  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X2 [KO:K03949]
100750797  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
100755602  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100770644  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100770579  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100773563  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100767451  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
100765100  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
100772543  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 isoform X2 [KO:K03956]
100757306  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100762893  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X2 [KO:K11353]
100754039  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X1 [KO:K03957]
100774095  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958]
100757431  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100762129  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100764614  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
100757916  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
100765445  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
100768089  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100754222  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100752642  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X2 [KO:K03964]
113830680  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X2 [KO:K03965]
100754681  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100689361  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
100754223  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
100768067  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
100761333  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100773999  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
100770445  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100751523  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
103160345  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X2 [KO:K03939]
100766693  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
100755890  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100766880  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100766442  Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100750732  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
100771246  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X2 [KO:K00235] [EC:1.3.5.1]
100689402  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
100774657  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
100759002  cytochrome b-c1 complex subunit Rieske, mitochondrial isoform X2 [KO:K00411] [EC:7.1.1.8]
3979182  CYTB; cytochrome b [KO:K00412]
100766032  cytochrome c1, heme protein, mitochondrial [KO:K00413]
100766805  cytochrome b-c1 complex subunit 1, mitochondrial isoform X2 [KO:K00414]
100750822  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100771461  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
100765588  cytochrome b-c1 complex subunit 6, mitochondrial isoform X2 [KO:K00416]
100762374  cytochrome b-c1 complex subunit 7 isoform X2 [KO:K00417]
100763175  cytochrome b-c1 complex subunit 8 isoform X2 [KO:K00418]
100774773  cytochrome b-c1 complex subunit 9 [KO:K00419]
100768142  cytochrome b-c1 complex subunit 10 [KO:K00420]
3979189  COX3; cytochrome c oxidase subunit III [KO:K02262]
3979185  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
3979186  COX2; cytochrome c oxidase subunit II [KO:K02261]
100770670  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
100757022  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
100765757  cytochrome c oxidase subunit 5A, mitochondrial isoform X2 [KO:K02264]
100768321  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
100764001  cytochrome c oxidase subunit 6A1, mitochondrial isoform X2 [KO:K02266]
100757101  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
100766809  cytochrome c oxidase subunit 6B1 [KO:K02267]
100768457  cytochrome c oxidase subunit 6B1 [KO:K02267]
113837268  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
103161269  cytochrome c oxidase subunit 6C-2 [KO:K02268]
100759281  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100756540  cytochrome c oxidase subunit 7A2, mitochondrial isoform X2 [KO:K02270]
100767351  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100756118  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
113837643  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
100772782  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
100764866  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
113834565  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100774785  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
103158861  cytochrome c oxidase subunit 7C, mitochondrial isoform X2 [KO:K02272]
100760019  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
100762701  cytochrome c oxidase subunit 8B, mitochondrial-like [KO:K02273]
100767292  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
100755207  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
100770431  Atp5f1a; ATP synthase subunit alpha, mitochondrial isoform X2 [KO:K02132]
100763024  Atp5f1b; ATP synthase subunit beta, mitochondrial isoform X2 [KO:K02133] [EC:7.1.2.2]
100760824  ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100766108  Atp5f1d; ATP synthase subunit delta, mitochondrial [KO:K02134]
100762406  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
103162776  Atp5f1e; ATP synthase subunit epsilon, mitochondrial isoform X2 [KO:K02135]
3979188  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100774535  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial isoform X2 [KO:K02127]
100772366  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
100752739  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100771114  ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100754380  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100755733  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
113831166  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100754287  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like isoform X2 [KO:K02128]
100771541  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
100770018  Atp5po; ATP synthase subunit O, mitochondrial isoform X2 [KO:K02137]
100756936  Atp5pf; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
3979187  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100765792  Vdac1; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1 isoform X2 [KO:K05862]
100750613  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
100752231  Tomm40; LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM40 homolog isoform X2 [KO:K11518]
100769989  Tomm40l; mitochondrial import receptor subunit TOM40B [KO:K11518]
100762628  Mcu; calcium uniporter protein, mitochondrial isoform X3 [KO:K20858]
100757303  Pink1; serine/threonine-protein kinase PINK1, mitochondrial isoform X2 [KO:K05688] [EC:2.7.11.1]
100764359  Prkn; E3 ubiquitin-protein ligase parkin isoform X2 [KO:K04556] [EC:2.3.2.31]
100773283  Optn; optineurin isoform X1 [KO:K19946]
100768352  Sqstm1; sequestosome-1 isoform X3 [KO:K14381]
100769810  Map1lc3b; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
100760576  Map1lc3a; microtubule-associated proteins 1A/1B light chain 3A isoform X2 [KO:K10435]
100757238  microtubule-associated proteins 1A/1B light chain 3B-like [KO:K10435]
100770091  serine/threonine-protein kinase TBK1 isoform X3 [KO:K05410] [EC:2.7.11.10]
100764984  Tank; TRAF family member-associated NF-kappa-B activator isoform X2 [KO:K12650]
100761254  Wdr41; WD repeat-containing protein 41 isoform X2 [KO:K23610]
100772792  Smcr8; guanine nucleotide exchange protein SMCR8 isoform X2 [KO:K23611]
100764363  C9orf72; guanine nucleotide exchange C9orf72 homolog isoform X1 [KO:K23609]
100760856  Rab8a; ras-related protein Rab-8A [KO:K07901]
100758199  Rab39b; ras-related protein Rab-39B [KO:K07925]
100752891  Rab1a; ras-related protein Rab-1A [KO:K07874]
100765911  LOW QUALITY PROTEIN: ras-related protein Rab-1A-like isoform X2 [KO:K07874]
100755421  Mtor; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
100772932  Ulk1; serine/threonine-protein kinase ULK1 isoform X2 [KO:K21357] [EC:2.7.11.1]
100759097  Ulk2; serine/threonine-protein kinase ULK2 isoform X3 [KO:K08269] [EC:2.7.11.1]
100763923  Atg101; autophagy-related protein 101 [KO:K19730]
100753698  Atg13; autophagy-related protein 13 isoform X1 [KO:K08331]
100763340  Rb1cc1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
100753101  Ambra1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
100751252  Becn2; beclin-2 [KO:K08334]
100762045  Becn1; beclin-1 isoform X2 [KO:K08334]
100769548  Pik3r4; phosphoinositide 3-kinase regulatory subunit 4 isoform X2 [KO:K08333] [EC:2.7.11.1]
100753411  Atg14; beclin 1-associated autophagy-related key regulator isoform X2 [KO:K17889]
100773882  Nrbf2; nuclear receptor-binding factor 2 isoform X2 [KO:K21246]
100765741  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X2 [KO:K00914] [EC:2.7.1.137]
100758391  Atg2a; autophagy-related protein 2 homolog A isoform X4 [KO:K17906]
100760198  Atg2b; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
100750963  Wipi1; WD repeat domain phosphoinositide-interacting protein 1 isoform X2 [KO:K17908]
100759282  Wipi2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
100763641  Als2; alsin isoform X1 [KO:K04575]
100772343  Rab5a; ras-related protein Rab-5A [KO:K07887]
100756792  Rac1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
113837706  ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
100773840  Chmp2b; charged multivesicular body protein 2b isoform X2 [KO:K12192]
100772123  Fig4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
100765400  Spg11; spatacsin isoform X4 [KO:K19026]
100751412  Ppp3cc; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X6 [KO:K04348] [EC:3.1.3.16]
100752571  serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X2 [KO:K04348] [EC:3.1.3.16]
100764987  Ppp3cb; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
100754314  Ppp3r2; calcineurin subunit B type 2 [KO:K06268]
100773706  Ppp3r1; calcineurin subunit B type 1 isoform X2 [KO:K06268]
100764107  Ubqln1; ubiquilin-1 isoform X2 [KO:K04523]
100763773  Ubqln4; ubiquilin-4 isoform X2 [KO:K04523]
100765357  Ubqln2; ubiquilin-2 isoform X2 [KO:K04523]
100763544  Ubqln3; ubiquilin-3 [KO:K04523]
103161970  Vcp; transitional endoplasmic reticulum ATPase isoform X2 [KO:K13525]
103161457  derlin-1 isoform X1 [KO:K11519]
100754440  Psma6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
100750876  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100756711  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
100769350  Psma7; proteasome subunit alpha type-7 isoform X2 [KO:K02731] [EC:3.4.25.1]
100769387  Psma8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
100774039  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100773907  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100758978  Psma3; proteasome subunit alpha type-3 isoform X2 [KO:K02727] [EC:3.4.25.1]
100769450  Psmb6; proteasome subunit beta type-6 isoform X2 [KO:K02738] [EC:3.4.25.1]
100771916  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100756232  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100767174  Psmb2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100769586  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100751285  Psmb1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100771179  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100757596  Psmc2; 26S proteasome regulatory subunit 7 isoform X2 [KO:K03061]
100770051  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
100766608  Psmc4; 26S proteasome regulatory subunit 6B isoform X2 [KO:K03063]
100762558  Psmc6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100689203  Psmc3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
100771099  Psmc5; 26S proteasome regulatory subunit 8 isoform X2 [KO:K03066]
100750425  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 isoform X2 [KO:K03028]
100760261  26S proteasome non-ATPase regulatory subunit 1 isoform X3 [KO:K03032]
100759692  26S proteasome non-ATPase regulatory subunit 3 isoform X3 [KO:K03033]
100768499  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
100767177  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100773369  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 isoform X2 [KO:K03036]
100751274  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100761962  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100772429  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 isoform X3 [KO:K03039]
103161668  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
100764392  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
100764857  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 isoform X2 [KO:K03031]
100754801  Adrm1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
100753658  Sem1; LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
100763688  Hdac6; histone deacetylase 6 [KO:K11407] [EC:3.5.1.98]
100769142  Tardbp; TAR DNA-binding protein 43 isoform X7 [KO:K23600]
100766288  LOW QUALITY PROTEIN: TAR DNA-binding protein 43-like [KO:K23600]
100756868  Fus; RNA-binding protein FUS isoform X1 [KO:K13098]
100769923  Hnrnpa1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
100757096  Hnrnpa3; heterogeneous nuclear ribonucleoprotein A3 isoform X2 [KO:K12741]
100759302  Hnrnpa2b1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X2 [KO:K13158]
100765372  Atxn2l; LOW QUALITY PROTEIN: ataxin-2-like protein isoform X13 [KO:K23625]
100772811  Atxn2; ataxin-2 isoform X3 [KO:K23625]
100755480  Matr3; matrin-3 isoform X1 [KO:K13213]
100755402  Ang; angiogenin [KO:K16631] [EC:3.1.27.-]
100770427  Ncbp1; nuclear cap-binding protein subunit 1 isoform X2 [KO:K12882]
100756310  Alyref; THO complex subunit 4 isoform X2 [KO:K12881]
100753039  Srsf3; serine/arginine-rich splicing factor 3 [KO:K12892]
100750467  serine/arginine-rich splicing factor 3-like [KO:K12892]
100770980  serine/arginine-rich splicing factor 3-like [KO:K12892]
100758380  Srsf7; serine/arginine-rich splicing factor 7 isoform X5 [KO:K12896]
100753177  Nxf1; nuclear RNA export factor 1 isoform X3 [KO:K14284]
100768937  Nxf3; LOW QUALITY PROTEIN: nuclear RNA export factor 3 [KO:K14284]
100770077  nuclear RNA export factor 5 isoform X2 [KO:K14284]
100768165  nuclear RNA export factor 2 isoform X1 [KO:K14284]
100765765  nuclear RNA export factor 2 [KO:K14284]
100761079  NTF2-related export protein 2 isoform X2 [KO:K14285]
100763994  Nxt1; NTF2-related export protein 1 [KO:K14285]
100767037  Gle1; nucleoporin GLE1 isoform X2 [KO:K18723]
100753776  Tpr; nucleoprotein TPR isoform X2 [KO:K09291]
100759187  Nup50; nuclear pore complex protein Nup50 isoform X2 [KO:K14295]
100761805  Nup153; nuclear pore complex protein Nup153 isoform X2 [KO:K14296]
100760516  Nup98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
100759605  Rae1; mRNA export factor isoform X1 [KO:K14298]
100759386  Sec13; protein SEC13 homolog [KO:K14004]
100757132  Seh1l; nucleoporin SEH1 isoform X2 [KO:K14299]
100770516  nucleoporin SEH1 isoform X2 [KO:K14299]
100757289  nucleoporin SEH1-like isoform X2 [KO:K14299]
100758918  LOW QUALITY PROTEIN: nucleoporin SEH1-like isoform X2 [KO:K14299]
113838934  nucleoporin SEH1-like [KO:K14299]
100773686  LOW QUALITY PROTEIN: nucleoporin SEH1-like isoform X2 [KO:K14299]
118237658  nucleoporin SEH1-like [KO:K14299]
118237634  LOW QUALITY PROTEIN: nucleoporin SEH1-like [KO:K14299]
100757678  LOW QUALITY PROTEIN: nucleoporin SEH1-like isoform X2 [KO:K14299]
100764532  LOW QUALITY PROTEIN: nucleoporin SEH1-like isoform X2 [KO:K14299]
113838745  nucleoporin SEH1-like [KO:K14299]
113837903  LOW QUALITY PROTEIN: nucleoporin SEH1-like [KO:K14299]
113838925  LOW QUALITY PROTEIN: nucleoporin SEH1-like [KO:K14299]
113838175  LOW QUALITY PROTEIN: nucleoporin SEH1-like [KO:K14299]
113837910  nucleoporin SEH1-like [KO:K14299]
113838462  LOW QUALITY PROTEIN: nucleoporin SEH1-like [KO:K14299]
118237660  nucleoporin SEH1-like [KO:K14299]
103159332  nucleoporin SEH1 [KO:K14299]
113838012  nucleoporin SEH1-like [KO:K14299]
113839002  nucleoporin SEH1-like [KO:K14299]
100768681  Nup133; nuclear pore complex protein Nup133 [KO:K14300]
100769827  Nup107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
100771280  Nup37; nucleoporin Nup37 [KO:K14302]
100765104  Nup160; nuclear pore complex protein Nup160 [KO:K14303]
100770676  Nup85; nuclear pore complex protein Nup85 isoform X2 [KO:K14304]
100758257  nuclear pore complex protein Nup85 isoform X6 [KO:K14304]
100758256  Nup43; nucleoporin Nup43 [KO:K14305]
100760998  Nup62cl; nucleoporin-62 C-terminal-like protein [KO:K14306]
100769030  Nup62; nuclear pore glycoprotein p62 [KO:K14306]
100763296  Nup58; nucleoporin p58/p45 isoform X1 [KO:K14307]
100770480  Nup54; nucleoporin p54 isoform X1 [KO:K14308]
100757790  Nup93; nuclear pore complex protein Nup93 [KO:K14309]
100773309  Nup205; nuclear pore complex protein Nup205 isoform X3 [KO:K14310]
100753702  nucleoporin NUP188 homolog isoform X4 [KO:K14311]
100772556  Nup155; nuclear pore complex protein Nup155 [KO:K14312]
100757207  Nup35; nucleoporin NUP35 [KO:K14313]
100765137  Nup210l; nuclear pore membrane glycoprotein 210-like isoform X1 [KO:K14314]
100772108  Nup210; nuclear pore membrane glycoprotein 210 isoform X2 [KO:K14314]
100766175  Ndc1; nucleoporin NDC1 isoform X1 [KO:K14315]
100767897  POM121-like protein 2 [KO:K14316]
100762026  Pom121c; nuclear envelope pore membrane protein POM 121C isoform X3 [KO:K14316]
100752558  Nup214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
100754367  Nup88; nuclear pore complex protein Nup88 isoform X2 [KO:K14318]
100762334  Ranbp2; E3 SUMO-protein ligase RanBP2 isoform X2 [KO:K12172] [EC:2.3.2.-]
100763725  Setx; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
100766619  Chchd10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
100689305  Hspa5; endoplasmic reticulum chaperone BiP precursor [KO:K09490] [EC:3.6.4.10]
100768207  Vapb; vesicle-associated membrane protein-associated protein B/C [KO:K10707]
100772173  Atf6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X2 [KO:K09054]
100763514  Ddit3; DNA damage-inducible transcript 3 protein isoform X2 [KO:K04452]
100750680  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X3 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100689062  Xbp1; X-box-binding protein 1 isoform 2 [KO:K09027]
100762099  Traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
100752376  Map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
100759746  Casp4; caspase-4 isoform X1 [KO:K04741] [EC:3.4.22.-]
100765343  Eif2ak3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X2 [KO:K08860] [EC:2.7.11.1]
100761915  Eif2s1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
100689386  Atf4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
100751775  inositol 1,4,5-trisphosphate receptor type 3 isoform X3 [KO:K04960]
100752825  Sigmar1; sigma non-opioid intracellular receptor 1 isoform X2 [KO:K20719]
100764780  Tnf; tumor necrosis factor isoform X1 [KO:K03156]
100765017  Tnfrsf1a; tumor necrosis factor receptor superfamily member 1A isoform X2 [KO:K03158]
100774542  Tnfrsf1b; tumor necrosis factor receptor superfamily member 1B isoform X2 [KO:K05141]
100774618  Daxx; death domain-associated protein 6 isoform X1 [KO:K02308]
100756793  Map2k3; dual specificity mitogen-activated protein kinase kinase 3 isoform X3 [KO:K04432] [EC:2.7.12.2]
100771767  dual specificity mitogen-activated protein kinase kinase 6 [KO:K04433] [EC:2.7.12.2]
100689104  Mapk14; mitogen-activated protein kinase 14 isoform X3 [KO:K04441] [EC:2.7.11.24]
100759172  Mapk11; mitogen-activated protein kinase 11 isoform X2 [KO:K04441] [EC:2.7.11.24]
100754042  Mapk12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
100763519  Mapk13; mitogen-activated protein kinase 13 isoform X2 [KO:K04441] [EC:2.7.11.24]
100758127  Nos1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
100757246  Ccs; copper chaperone for superoxide dismutase [KO:K04569]
100766810  Gpx5; epididymal secretory glutathione peroxidase precursor [KO:K00432] [EC:1.11.1.9]
100756109  Gpx7; glutathione peroxidase 7 precursor [KO:K00432] [EC:1.11.1.9]
100771188  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
100751356  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100764638  Gpx3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
100766519  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
100775000  Gpx8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
100773774  Cat; catalase isoform X1 [KO:K03781] [EC:1.11.1.6]
100768595  Prph; peripherin isoform X1 [KO:K07607]
100774151  Nefl; neurofilament light polypeptide [KO:K04572]
100773854  Nefm; neurofilament medium polypeptide [KO:K04573]
100753127  Nefh; neurofilament heavy polypeptide isoform X2 [KO:K04574]
100757165  profilin-2 [KO:K05759]
100760079  Pfn4; profilin-4 [KO:K05759]
100771269  Pfn3; profilin-3 [KO:K05759]
100753478  Pfn1; profilin-1 isoform X2 [KO:K05759]
100770381  Pfn2; profilin-2 isoform X2 [KO:K05759]
113830633  Actg1; actin, cytoplasmic 2 isoform X2 [KO:K05692]
100689477  Actb; actin, cytoplasmic 1 [KO:K05692]
100763423  Anxa7; annexin A7 isoform X1 [KO:K17095]
100752736  Anxa11; annexin A11 isoform X1 [KO:K17095]
113836595  LOW QUALITY PROTEIN: annexin A7-like [KO:K17095]
100774173  Kif5b; kinesin-1 heavy chain isoform X2 [KO:K10396]
100763800  Kif5a; kinesin heavy chain isoform X2 [KO:K10396]
100773258  Kif5c; kinesin heavy chain isoform X2 [KO:K10396]
100774201  Klc4; kinesin light chain 4 [KO:K10407]
100764190  Klc2; kinesin light chain 2 [KO:K10407]
100689219  Klc1; kinesin light chain 1 isoform X7 [KO:K10407]
113837894  kinesin light chain 3 isoform X3 [KO:K10407]
113837287  kinesin light chain 3 isoform X2 [KO:K10407]
100682526  Tuba1c; tubulin alpha-1C chain [KO:K07374]
100682527  Tuba1a; tubulin alpha-1A chain [KO:K07374]
100682528  Tuba1b; tubulin alpha-1B chain [KO:K07374]
113832602  tubulin alpha-1C chain-like [KO:K07374]
100767686  Tuba4a; tubulin alpha-4A chain isoform X1 [KO:K07374]
100755907  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
100767630  tubulin alpha chain [KO:K07374]
100765599  tubulin alpha-8 chain isoform X2 [KO:K07374]
100762888  tubulin alpha-3 chain [KO:K07374]
100753526  Tuba8; tubulin alpha-8 chain isoform X1 [KO:K07374]
100772797  Tubb1; tubulin beta-1 chain isoform X2 [KO:K07375]
100760055  tubulin beta-3 chain isoform X2 [KO:K07375]
100689091  Tubb; tubulin beta-5 chain [KO:K07375]
100761889  tubulin beta-2A chain isoform X6 [KO:K07375]
100689363  Tubb6; tubulin beta-6 chain [KO:K07375]
100763390  Tubb4a; tubulin beta-4A chain isoform X2 [KO:K07375]
100689089  Tubb4b; tubulin beta-4B chain [KO:K07375]
100758633  tubulin beta-2B chain isoform X4 [KO:K07375]
100765083  Dctn1; dynactin subunit 1 isoform X2 [KO:K04648]
100757525  Dctn2; dynactin subunit 2 isoform X1 [KO:K10424]
100753423  Dctn3; dynactin subunit 3 isoform X3 [KO:K10425]
100767865  Dctn4; dynactin subunit 4 isoform X2 [KO:K10426]
100773966  dynactin subunit 5 isoform X3 [KO:K10427]
100754783  Dctn6; dynactin subunit 6 [KO:K10428]
100750704  Actr1a; alpha-centractin isoform X1 [KO:K16575]
100768031  Actr1b; beta-centractin isoform X2 [KO:K16575]
100759261  Actr10; actin-related protein 10 [KO:K16576]
100757326  Hap1; huntingtin-associated protein 1 [KO:K04647]
100752773  Dnah7; dynein heavy chain 7, axonemal isoform X2 [KO:K10408]
100771964  Dnah5; dynein heavy chain 5, axonemal isoform X2 [KO:K10408]
100754128  Dnah6; dynein heavy chain 6, axonemal isoform X2 [KO:K10408]
100770690  Dnah9; dynein heavy chain 9, axonemal isoform X2 [KO:K10408]
100770442  Dnah11; dynein heavy chain 11, axonemal isoform X2 [KO:K10408]
100750737  Dnah12; dynein heavy chain 12, axonemal isoform X4 [KO:K10408]
100763108  Dnah8; dynein heavy chain 8, axonemal isoform X2 [KO:K10408]
103161549  Dnah10; dynein heavy chain 10, axonemal isoform X2 [KO:K10408]
100768695  Dnah17; LOW QUALITY PROTEIN: dynein heavy chain 17, axonemal isoform X3 [KO:K10408]
100752669  Dnah14; LOW QUALITY PROTEIN: dynein heavy chain 14, axonemal isoform X2 [KO:K10408]
100765769  Dnah1; dynein heavy chain 1, axonemal [KO:K10408]
100752771  Dnah3; dynein heavy chain 3, axonemal isoform X2 [KO:K10408]
100756503  Dnah2; dynein heavy chain 2, axonemal isoform X2 [KO:K10408]
100754224  Dnali1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
100754021  Dnai1; dynein intermediate chain 1, axonemal isoform X3 [KO:K10409]
100761698  Dnai2; dynein intermediate chain 2, axonemal [KO:K11143]
100774309  Dnal1; dynein light chain 1, axonemal isoform X2 [KO:K10411]
100772767  Dnal4; dynein light chain 4, axonemal [KO:K10412]
100752285  Slc1a2; excitatory amino acid transporter 2 [KO:K05613]
100760062  Nos2; nitric oxide synthase, inducible isoform X2 [KO:K13241] [EC:1.14.13.39]
100769392  Gria1; glutamate receptor 1 isoform X2 [KO:K05197]
100764344  Gria2; glutamate receptor 2 isoform X2 [KO:K05198]
100770880  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
100764315  Grin2a; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
118239276  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100758804  Grin2c; glutamate receptor ionotropic, NMDA 2C isoform X3 [KO:K05211]
100750615  Grin2d; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D isoform X2 [KO:K05212]
100757907  Nrg1; pro-neuregulin-1, membrane-bound isoform isoform X9 [KO:K05455]
100759245  Nrg2; LOW QUALITY PROTEIN: pro-neuregulin-2, membrane-bound isoform isoform X2 [KO:K05456]
100763774  Nrg3; pro-neuregulin-3, membrane-bound isoform isoform X2 [KO:K05457]
100757283  Nrg4; pro-neuregulin-4, membrane-bound isoform [KO:K05458]
100760475  receptor tyrosine-protein kinase erbB-4 isoform X2 [KO:K05085] [EC:2.7.10.1]
Compound
C00001  H2O
C00008  ADP
C00025  L-Glutamate
C00027  Hydrogen peroxide
C00039  DNA
C00046  RNA
C00062  L-Arginine
C00070  Copper
C00076  Calcium cation
C00533  Nitric oxide
C00704  Superoxide
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
C16844  Hydroxyl radical
C16845  Peroxynitrite
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DOI:10.1111/j.1582-4934.2011.01374.x
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Turner BJ, Atkin JD
  Title
ER stress and UPR in familial amyotrophic lateral sclerosis.
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Curr Mol Med 6:79-86 (2006)
DOI:10.2174/156652406775574550
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Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, Noguchi T, Matsuzawa A, Takeda K, Ichijo H
  Title
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
  Journal
Genes Dev 22:1451-64 (2008)
DOI:10.1101/gad.1640108
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Walker AK, Atkin JD
  Title
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
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IUBMB Life 63:754-63 (2011)
DOI:10.1002/iub.520
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Renaud L, Picher-Martel V, Codron P, Julien JP
  Title
Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
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Acta Neuropathol Commun 7:103 (2019)
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Nguyen HP, Van Broeckhoven C, van der Zee J
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ALS Genes in the Genomic Era and their Implications for FTD.
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Trends Genet 34:404-423 (2018)
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Atkin G, Paulson H
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Ubiquitin pathways in neurodegenerative disease.
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Front Mol Neurosci 7:63 (2014)
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Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
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Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1(G93A) Rodent Spinal Cord Tissue.
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Neurochem Res 44:228-233 (2019)
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Benarroch EE
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Sigma-1 receptor and amyotrophic lateral sclerosis.
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Neurology 91:743-747 (2018)
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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)
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Related
pathway
cge00190  Oxidative phosphorylation
cge03013  Nucleocytoplasmic transport
cge03050  Proteasome
cge04010  MAPK signaling pathway
cge04012  ErbB signaling pathway
cge04020  Calcium signaling pathway
cge04115  p53 signaling pathway
cge04137  Mitophagy - animal
cge04140  Autophagy - animal
cge04141  Protein processing in endoplasmic reticulum
cge04210  Apoptosis
cge04724  Glutamatergic synapse
cge04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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