KEGG   PATHWAY: oor05014
Entry
oor05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Orcinus orca (killer whale)
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
oor05014  Amyotrophic lateral sclerosis
oor05014

Organism
Orcinus orca (killer whale) [GN:oor]
Gene
101285670  TP53; cellular tumor antigen p53 isoform X2 [KO:K04451]
101289850  CASP1; caspase-1 [KO:K01370] [EC:3.4.22.36]
101277745  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
101276750  BCL2; apoptosis regulator Bcl-2 [KO:K02161]
101288063  BCL2L1; bcl-2-like protein 1 isoform X1 [KO:K04570]
101285349  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
101274967  BAD; bcl2-associated agonist of cell death [KO:K02158]
101280303  APAF1; apoptotic protease-activating factor 1 isoform X4 [KO:K02084]
105747906  cytochrome c-like [KO:K08738]
101283052  cytochrome c [KO:K08738]
101275631  cytochrome c-like [KO:K08738]
105747703  cytochrome c 2 [KO:K08738]
101272869  cytochrome c-like [KO:K08738]
117203203  LOW QUALITY PROTEIN: cytochrome c-like [KO:K08738]
101271677  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
101289847  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
101280711  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
72909410  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
72909413  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
72909422  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
72909401  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
72909423  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
72909426  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
72909403  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
101289424  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
101274683  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
101280712  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X3 [KO:K03944]
117202624  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
101290492  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
101278388  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
101280277  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101275613  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
101279080  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
117197517  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
117199347  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
117203566  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
101282532  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101272964  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
101289975  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951]
101276332  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
101276458  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X2 [KO:K03953]
101269174  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
101275506  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
101276536  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
101287214  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
101288751  NDUFA13; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
101288261  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
101281008  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
101277437  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
101278952  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
101282167  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
101282935  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
101284850  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
101289993  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
101276092  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
101283098  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
101269570  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
101282465  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X3 [KO:K03964]
101277485  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
101279385  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
101276209  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
101280311  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial-like [KO:K11351]
101269912  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
101277400  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
101275970  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
101281506  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
101288800  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
101277019  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
101289305  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X7 [KO:K03940] [EC:7.1.1.2]
101269404  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
101284218  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101271821  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
101290330  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
101277306  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
101271279  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
101278085  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101275820  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
101275408  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
101277363  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
101271681  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
101274086  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
72909427  CYTB; cytochrome b [KO:K00412]
101282531  cytochrome c1, heme protein, mitochondrial [KO:K00413]
101286248  cytochrome b-c1 complex subunit 1, mitochondrial isoform X4 [KO:K00414]
101269781  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
101279534  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
101282996  cytochrome b-c1 complex subunit 7 [KO:K00417]
101271752  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101289974  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101271385  cytochrome b-c1 complex subunit 8 [KO:K00418]
125960973  cytochrome b-c1 complex subunit 8-like [KO:K00418]
101282440  cytochrome b-c1 complex subunit 9 [KO:K00419]
101276993  cytochrome b-c1 complex subunit 10 [KO:K00420]
101287995  cytochrome b-c1 complex subunit 10-like [KO:K00420]
117202030  cytochrome b-c1 complex subunit 10-like [KO:K00420]
72909398  COX3; cytochrome c oxidase subunit III [KO:K02262]
72909418  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
72909420  COX2; cytochrome c oxidase subunit II [KO:K02261]
101287815  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
101278353  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
101289495  cytochrome c oxidase subunit 5A, mitochondrial isoform X3 [KO:K02264]
101287413  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
101289884  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
101288308  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
101282104  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
101289435  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
101274306  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6B2 [KO:K02267]
101289868  cytochrome c oxidase subunit 6B1 [KO:K02267]
117202988  cytochrome c oxidase subunit 6C-like [KO:K02268]
117203493  cytochrome c oxidase subunit 6C [KO:K02268]
101287635  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
117199478  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
101275626  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
101278311  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
101272241  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
117197361  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
117198668  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
117201473  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
117201652  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
105748355  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
117198471  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
117200905  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
117203191  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
101273443  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
101279474  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
101280534  LOW QUALITY PROTEIN: ATP synthase subunit beta, mitochondrial-like [KO:K02133] [EC:7.1.2.2]
101278268  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
101290442  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
117197667  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
117198797  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
72909397  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
101286579  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
101287179  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
101281341  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
101286239  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
101277818  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
101288920  ATP5PD; ATP synthase subunit d, mitochondrial isoform X2 [KO:K02138]
101286843  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
101270250  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
72909421  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
101289999  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
101276658  TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518]
101270786  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
101270140  MCU; calcium uniporter protein, mitochondrial isoform X3 [KO:K20858]
101287390  PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
101275093  optineurin [KO:K19946]
101275366  OPTN; optineurin isoform X1 [KO:K19946]
101286416  SQSTM1; sequestosome-1 [KO:K14381]
101279469  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
101276622  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
101277730  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
101290315  TBK1; serine/threonine-protein kinase TBK1 [KO:K05410] [EC:2.7.11.10]
101275571  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
101279463  WDR41; WD repeat-containing protein 41 isoform X2 [KO:K23610]
101281225  SMCR8; guanine nucleotide exchange protein SMCR8 [KO:K23611]
101280918  C9orf72; guanine nucleotide exchange factor C9orf72 homolog isoform X1 [KO:K23609]
101274200  RAB8A; ras-related protein Rab-8A [KO:K07901]
101286345  RAB39B; ras-related protein Rab-39B [KO:K07925]
101284611  RAB1A; ras-related protein Rab-1A [KO:K07874]
101284249  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
101281996  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
101285840  ULK2; serine/threonine-protein kinase ULK2 isoform X2 [KO:K08269] [EC:2.7.11.1]
101273125  ATG101; autophagy-related protein 101 [KO:K19730]
101288098  ATG13; autophagy-related protein 13 isoform X4 [KO:K08331]
101284338  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
101287189  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X2 [KO:K17985]
101287436  BECN1; beclin-1 isoform X3 [KO:K08334]
101278206  BECN2; beclin-2 [KO:K08334]
101282029  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
101273974  ATG14; beclin 1-associated autophagy-related key regulator isoform X1 [KO:K17889]
101274619  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101277227  nuclear receptor-binding factor 2-like [KO:K21246]
101273072  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X2 [KO:K00914] [EC:2.7.1.137]
101277378  ATG2B; autophagy-related protein 2 homolog B isoform X2 [KO:K17906]
101281629  ATG2A; autophagy-related protein 2 homolog A isoform X3 [KO:K17906]
101279066  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
101278800  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X3 [KO:K17908]
101278761  ALS2; alsin isoform X1 [KO:K04575]
101273864  RAB5A; ras-related protein Rab-5A isoform X1 [KO:K07887]
101275059  ras-related protein Rab-5A-like [KO:K07887]
101274018  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X3 [KO:K04392]
101281122  charged multivesicular body protein 2b [KO:K12192]
101272760  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
101284291  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
101284416  SPG11; spatacsin isoform X4 [KO:K19026]
101271019  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X7 [KO:K04348] [EC:3.1.3.16]
101278517  PPP3CA; protein phosphatase 3 catalytic subunit alpha isoform X3 [KO:K04348] [EC:3.1.3.16]
101286888  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X7 [KO:K04348] [EC:3.1.3.16]
105748080  PPP3R2; LOW QUALITY PROTEIN: calcineurin subunit B type 2 [KO:K06268]
101272877  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
101285249  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
101273560  UBQLN3; ubiquilin-3 [KO:K04523]
101284472  UBQLN4; ubiquilin-4 isoform X3 [KO:K04523]
101277544  UBQLN2; ubiquilin-2 [KO:K04523]
101289076  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
101281053  DERL1; derlin-1 isoform X2 [KO:K11519]
101289069  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
101272302  PSMA2; proteasome subunit alpha type-2 isoform X1 [KO:K02726] [EC:3.4.25.1]
101276738  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
101271434  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
101289439  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
101275300  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
101271046  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
101269411  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
101284646  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
101271178  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101276891  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
101280817  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
101282028  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
101270235  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
101279448  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
101287767  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
101290377  26S proteasome regulatory subunit 7-like [KO:K03061]
101285119  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
101273535  PSMC4; 26S proteasome regulatory subunit 6B isoform X2 [KO:K03063]
101281278  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
101274076  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
101290309  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
101273655  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
101285485  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
101286324  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 isoform X2 [KO:K03033]
101278004  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
101284178  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
101288044  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
101272672  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
101271432  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
101288168  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
101277171  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
101275060  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
101276816  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
101279254  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
101283086  TARDBP; TAR DNA-binding protein 43 [KO:K23600]
101286694  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
101281565  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
101288330  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
101274438  heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
101289249  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
101269526  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
101271809  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
101274488  HNRNPA1L3; LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like 3 [KO:K12741]
101277757  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105748200  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
101277422  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
101286082  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
117201966  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
101283805  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X3 [KO:K13158]
101270382  heterogeneous nuclear ribonucleoproteins A2/B1-like isoform X1 [KO:K13158]
117202385  heterogeneous nuclear ribonucleoproteins A2/B1-like isoform X2 [KO:K13158]
101285683  ATXN2L; ataxin-2-like protein isoform X11 [KO:K23625]
101288164  ATXN2; ataxin-2 isoform X5 [KO:K23625]
101284026  MATR3; matrin-3 isoform X1 [KO:K13213]
101273483  LOW QUALITY PROTEIN: matrin-3-like [KO:K13213]
101270637  ANG; angiogenin isoform X2 [KO:K16631] [EC:3.1.27.-]
101283834  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
101275531  ALYREF; THO complex subunit 4 [KO:K12881]
101288810  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
101285243  serine/arginine-rich splicing factor 3-like [KO:K12892]
101287781  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
101286095  nuclear RNA export factor 3 isoform X1 [KO:K14284]
101287280  LOW QUALITY PROTEIN: nuclear RNA export factor 2 [KO:K14284]
101284211  NXF1; nuclear RNA export factor 1 isoform X4 [KO:K14284]
101287516  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
101276877  NXT2; NTF2-related export protein 2 isoform X2 [KO:K14285]
101285083  NXT1; NTF2-related export protein 1 [KO:K14285]
101271014  GLE1; mRNA export factor GLE1 [KO:K18723]
101290053  TPR; nucleoprotein TPR isoform X1 [KO:K09291]
101280679  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
101272265  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
101287256  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
101283867  RAE1; mRNA export factor RAE1 isoform X1 [KO:K14298]
101273873  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
101279994  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
101286065  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
101285486  NUP107; nuclear pore complex protein Nup107 isoform X2 [KO:K14301]
101271179  NUP37; nucleoporin Nup37 [KO:K14302]
101276980  NUP160; nuclear pore complex protein Nup160 isoform X1 [KO:K14303]
101269644  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
101282774  NUP43; nucleoporin Nup43 [KO:K14305]
101289307  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
101274867  NUP62CL; nucleoporin-62 C-terminal-like protein isoform X1 [KO:K14306]
101282604  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
101288176  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
101276733  NUP93; nuclear pore complex protein Nup93 isoform X5 [KO:K14309]
101285860  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
101288726  NUP188; nucleoporin NUP188 isoform X1 [KO:K14311]
101271735  NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
101273521  NUP35; nucleoporin NUP35 isoform X1 [KO:K14313]
101274979  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
101279952  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
101276341  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
101285258  POM121C; nuclear envelope pore membrane protein POM 121C isoform X1 [KO:K14316]
101287039  LOW QUALITY PROTEIN: POM121-like protein 2 [KO:K14316]
101272190  LOW QUALITY PROTEIN: POM121-like protein 2 [KO:K14316]
101279973  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
101287875  NUP88; nuclear pore complex protein Nup88 [KO:K14318]
101272377  RGPD4; ranBP2-like and GRIP domain-containing protein 4 isoform X1 [KO:K12172] [EC:2.3.2.-]
101280829  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
101274533  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
101289151  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
101285386  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
101284204  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
101278090  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
101288419  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101288279  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
101281303  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
101277232  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
101272972  caspase-12 isoform X1 [KO:K04741] [EC:3.4.22.-]
101282451  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
101281288  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
101283438  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
101280894  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
101271687  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
101269870  TNF; tumor necrosis factor [KO:K03156]
101282502  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
101289721  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X2 [KO:K05141]
101283649  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
101275666  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X2 [KO:K04432] [EC:2.7.12.2]
101280413  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
101269212  MAPK13; mitogen-activated protein kinase 13 isoform X7 [KO:K04441] [EC:2.7.11.24]
101269458  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
101287579  mitogen-activated protein kinase 12 isoform X6 [KO:K04441] [EC:2.7.11.24]
101287833  mitogen-activated protein kinase 11 isoform X5 [KO:K04441] [EC:2.7.11.24]
101273057  NOS1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
101278921  CCS; copper chaperone for superoxide dismutase isoform X2 [KO:K04569]
101289592  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
101286782  glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
101280581  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
101277951  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
101273590  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
101284176  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
101272225  CAT; catalase [KO:K03781] [EC:1.11.1.6]
101288914  PRPH; peripherin isoform X3 [KO:K07607]
101277008  NEFL; neurofilament light polypeptide [KO:K04572]
101276760  NEFM; neurofilament medium polypeptide [KO:K04573]
101284970  NEFH; neurofilament heavy polypeptide [KO:K04574]
101281814  PFN4; profilin-4 isoform X1 [KO:K05759]
101285162  uncharacterized protein LOC101285162 [KO:K05759]
101277487  PFN3; profilin-3 [KO:K05759]
101285419  PFN1; profilin-1 [KO:K05759]
101286818  PFN2; profilin-2 isoform X2 [KO:K05759]
101282200  profilin-1-like [KO:K05759]
101271282  ACTG1; actin, cytoplasmic 2 [KO:K05692]
101277826  ACTB; actin, cytoplasmic 1 [KO:K05692]
101290389  ANXA11; annexin A11 isoform X1 [KO:K17095]
101287559  ANXA7; annexin A7 isoform X1 [KO:K17095]
101284316  KIF5C; kinesin heavy chain [KO:K10396]
101288686  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
101279321  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
101288570  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
101277152  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
101270328  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
101288559  KLC4; kinesin light chain 4 [KO:K10407]
101289814  TUBA1B; tubulin alpha-1B chain isoform X1 [KO:K07374]
101289167  tubulin alpha-1C chain [KO:K07374]
101283945  tubulin alpha-1D chain-like [KO:K07374]
101284203  TUBA4A; tubulin alpha-4A chain [KO:K07374]
101290059  tubulin alpha-3 chain [KO:K07374]
101269236  tubulin alpha-3 chain [KO:K07374]
101276235  TUBA8; tubulin alpha-8 chain [KO:K07374]
101281777  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
101289567  tubulin alpha-1A chain [KO:K07374]
101271582  TUBB; tubulin beta chain [KO:K07375]
101269831  TUBB3; tubulin beta-3 chain isoform X1 [KO:K07375]
101277695  TUBB6; tubulin beta-6 chain [KO:K07375]
101280329  TUBB4A; tubulin beta-4A chain [KO:K07375]
101275905  TUBB4B; tubulin beta-4B chain [KO:K07375]
101274301  tubulin beta chain-like [KO:K07375]
101287587  TUBB1; tubulin beta-1 chain [KO:K07375]
101289275  tubulin beta-2B chain isoform X2 [KO:K07375]
101271784  DCTN1; dynactin subunit 1 isoform X8 [KO:K04648]
101278579  DCTN2; dynactin subunit 2 isoform X4 [KO:K10424]
101270886  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
101290332  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
101275996  DCTN5; dynactin subunit 5 [KO:K10427]
101289178  DCTN6; dynactin subunit 6 [KO:K10428]
101289845  dynactin subunit 6-like [KO:K10428]
101274015  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
101287165  ACTR1B; beta-centractin [KO:K16575]
101269828  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
101276560  HAP1; huntingtin-associated protein 1 [KO:K04647]
101282519  DNAH11; LOW QUALITY PROTEIN: dynein axonemal heavy chain 11 [KO:K10408]
101273543  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
101288926  DNAH10; dynein axonemal heavy chain 10 isoform X1 [KO:K10408]
101271246  DNAH9; dynein axonemal heavy chain 9 isoform X1 [KO:K10408]
101289887  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
101287977  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
101283315  DNAH8; dynein axonemal heavy chain 8 isoform X1 [KO:K10408]
101285105  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
101283980  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
101284899  DNAH2; dynein axonemal heavy chain 2 [KO:K10408]
101283317  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
101274363  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
101273252  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
101269561  DNAI1; dynein axonemal intermediate chain 1 [KO:K10409]
101283246  DNAI2; LOW QUALITY PROTEIN: dynein axonemal intermediate chain 2 [KO:K11143]
101277131  DNAL1; dynein axonemal light chain 1 isoform X2 [KO:K10411]
101280857  DNAL4; dynein axonemal light chain 4 isoform X4 [KO:K10412]
101275648  SLC1A2; excitatory amino acid transporter 2 isoform X2 [KO:K05613]
101280638  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
101289103  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
101277565  GRIA2; glutamate receptor 2 isoform X4 [KO:K05198]
101278622  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X4 [KO:K05208]
101270526  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
101285554  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101286467  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X4 [KO:K05211]
101270626  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
101276475  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X2 [KO:K05455]
101274058  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
101287317  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X4 [KO:K05457]
101275944  NRG4; pro-neuregulin-4, membrane-bound isoform [KO:K05458]
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
Reference
PMID:16472115
  Authors
Turner BJ, Atkin JD
  Title
ER stress and UPR in familial amyotrophic lateral sclerosis.
  Journal
Curr Mol Med 6:79-86 (2006)
DOI:10.2174/156652406775574550
Reference
PMID:18519638
  Authors
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
Reference
PMID:21834058
  Authors
Walker AK, Atkin JD
  Title
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
  Journal
IUBMB Life 63:754-63 (2011)
DOI:10.1002/iub.520
Reference
PMID:31319884
  Authors
Renaud L, Picher-Martel V, Codron P, Julien JP
  Title
Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
  Journal
Acta Neuropathol Commun 7:103 (2019)
DOI:10.1186/s40478-019-0758-7
Reference
PMID:29605155
  Authors
Nguyen HP, Van Broeckhoven C, van der Zee J
  Title
ALS Genes in the Genomic Era and their Implications for FTD.
  Journal
Trends Genet 34:404-423 (2018)
DOI:10.1016/j.tig.2018.03.001
Reference
PMID:25071440
  Authors
Atkin G, Paulson H
  Title
Ubiquitin pathways in neurodegenerative disease.
  Journal
Front Mol Neurosci 7:63 (2014)
DOI:10.3389/fnmol.2014.00063
Reference
PMID:29299811
  Authors
Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
  Title
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.
  Journal
Neurochem Res 44:228-233 (2019)
DOI:10.1007/s11064-017-2461-z
Reference
PMID:30242016
  Authors
Benarroch EE
  Title
Sigma-1 receptor and amyotrophic lateral sclerosis.
  Journal
Neurology 91:743-747 (2018)
DOI:10.1212/WNL.0000000000006347
Reference
PMID:23361386
  Authors
Millecamps S, Julien JP
  Title
Axonal transport deficits and neurodegenerative diseases.
  Journal
Nat Rev Neurosci 14:161-76 (2013)
DOI:10.1038/nrn3380
Related
pathway
oor00190  Oxidative phosphorylation
oor03013  Nucleocytoplasmic transport
oor03050  Proteasome
oor04010  MAPK signaling pathway
oor04012  ErbB signaling pathway
oor04020  Calcium signaling pathway
oor04115  p53 signaling pathway
oor04137  Mitophagy - animal
oor04140  Autophagy - animal
oor04141  Protein processing in endoplasmic reticulum
oor04210  Apoptosis
oor04724  Glutamatergic synapse
oor04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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