KEGG   PATHWAY: mmyo05014
Entry
mmyo05014                   Pathway                                
Name
Amyotrophic lateral sclerosis - Myotis myotis (greater mouse-eared bat)
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
mmyo05014  Amyotrophic lateral sclerosis
mmyo05014

Organism
Myotis myotis (greater mouse-eared bat) [GN:mmyo]
Gene
118672084  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
118652071  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
118663765  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
118663066  BCL2L1; bcl-2-like protein 1 isoform X1 [KO:K04570]
118655437  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
118665363  BAD; bcl2-associated agonist of cell death [KO:K02158]
118650180  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
118666111  cytochrome c [KO:K08738]
118679308  cytochrome c-like [KO:K08738]
118654943  cytochrome c-like [KO:K08738]
118677148  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
118650210  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
118674974  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
26889517  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
26889520  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
26889529  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
26889508  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
26889530  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
26889533  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
26889510  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
118665210  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
118663528  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
118676864  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
118658680  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
118653714  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
118653841  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
118659276  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
118651524  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
118666177  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
118659978  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
118666453  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
118674353  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6-like [KO:K03950]
118651854  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
118659198  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
118667132  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
118650282  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
118677633  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial-like [KO:K03953]
118661392  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
118656414  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
118658957  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
118651305  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
118658191  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
118659585  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
118666341  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
118661238  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
118676920  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
118676944  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
118667403  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
118652705  NDUFB7; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
118669847  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
118670981  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
118656353  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
118653795  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
118661169  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
118672885  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
118665049  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
118656177  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
118656173  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
118658662  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
118665205  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
118659095  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
118664595  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
118655921  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
118670565  succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial-like [KO:K00235] [EC:1.3.5.1]
118676311  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
118672944  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
118664349  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
118674548  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
26889534  CYTB; cytochrome b [KO:K00412]
118658381  cytochrome c1, heme protein, mitochondrial-like [KO:K00413]
118653082  cytochrome c1, heme protein, mitochondrial [KO:K00413]
118676147  cytochrome c1, heme protein, mitochondrial-like [KO:K00413]
118668510  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
118656194  cytochrome b-c1 complex subunit 2, mitochondrial isoform X1 [KO:K00415]
118677282  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
118658665  cytochrome b-c1 complex subunit 7-like [KO:K00417]
118670644  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
118658647  cytochrome b-c1 complex subunit 8 [KO:K00418]
118678367  cytochrome b-c1 complex subunit 9 [KO:K00419]
118678145  cytochrome b-c1 complex subunit 10-like [KO:K00420]
118658907  cytochrome b-c1 complex subunit 10 [KO:K00420]
26889505  COX3; cytochrome c oxidase subunit III [KO:K02262]
26889525  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
26889527  COX2; cytochrome c oxidase subunit II [KO:K02261]
118674112  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
118663651  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
118675843  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
118676017  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
118652480  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
118678114  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
118656385  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
118657409  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
118666421  cytochrome c oxidase subunit 6B1-like [KO:K02267]
118669352  cytochrome c oxidase subunit 6B1-like [KO:K02267]
118655199  cytochrome c oxidase subunit 6B2 [KO:K02267]
118674654  cytochrome c oxidase subunit 6B1 [KO:K02267]
118670671  cytochrome c oxidase subunit 6C [KO:K02268]
118671315  cytochrome c oxidase subunit 6C-like [KO:K02268]
118674194  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
118660137  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
118669062  uncharacterized protein LOC118669062 isoform X1 [KO:K02270]
118651610  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
118654460  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
118656735  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
118665381  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
118662999  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
118650906  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
118672118  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
118649386  ATP synthase subunit delta, mitochondrial-like [KO:K02134]
118659348  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
118662963  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
118672074  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
26889504  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
118672708  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
118651236  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
118657855  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
118661898  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
118663441  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
118670790  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
118671931  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
118665670  ATP synthase subunit d, mitochondrial-like [KO:K02138]
118671446  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
118676997  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
118655681  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
118675387  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
26889528  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
118658112  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
118673109  TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518]
118674270  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
118654015  LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
118670134  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
118677738  PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
118660195  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
118677446  OPTN; optineurin isoform X1 [KO:K19946]
118658615  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
118674235  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B isoform X1 [KO:K10435]
118662561  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
118651925  TBK1; serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
118661088  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
118656083  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
118649395  SMCR8; guanine nucleotide exchange protein SMCR8 [KO:K23611]
118667610  C9orf72; guanine nucleotide exchange C9orf72 homolog isoform X1 [KO:K23609]
118652600  RAB8A; ras-related protein Rab-8A [KO:K07901]
118676521  LOW QUALITY PROTEIN: ras-related protein Rab-8A-like [KO:K07901]
118654697  RAB39B; ras-related protein Rab-39B [KO:K07925]
118668936  RAB1A; ras-related protein Rab-1A isoform X1 [KO:K07874]
118675322  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
118653012  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
118649389  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
118651135  ATG101; autophagy-related protein 101 isoform X1 [KO:K19730]
118665077  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
118670959  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
118665082  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
118678960  beclin-2-like [KO:K08334]
118671466  BECN1; beclin-1 isoform X1 [KO:K08334]
118676302  beclin-2-like [KO:K08334]
118668185  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
118655744  ATG14; beclin 1-associated autophagy-related key regulator isoform X1 [KO:K17889]
118670051  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
118662908  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
118660375  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
118665340  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
118657857  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
118667386  LOW QUALITY PROTEIN: WD repeat domain phosphoinositide-interacting protein 2-like [KO:K17908]
118671561  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
118661151  ALS2; alsin isoform X1 [KO:K04575]
118668224  RAB5A; ras-related protein Rab-5A isoform X1 [KO:K07887]
118657696  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
118675378  CHMP2B; charged multivesicular body protein 2b isoform X1 [KO:K12192]
118660230  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
118651090  SPG11; spatacsin isoform X1 [KO:K19026]
118658346  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118670147  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118671846  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118667234  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
118668920  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
118667426  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
118653998  UBQLN2; ubiquilin-2 [KO:K04523]
118663134  UBQLN3; ubiquilin-3 [KO:K04523]
118672847  ubiquilin-4 isoform X1 [KO:K04523]
118667356  transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
118670813  DERL1; derlin-1 [KO:K11519]
118677907  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
118667652  proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
118665958  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
118674683  proteasome subunit alpha type-2-like [KO:K02726] [EC:3.4.25.1]
118679096  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
118663715  PSMA7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
118663798  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
118672988  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
118676281  LOW QUALITY PROTEIN: proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
118664860  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
118656008  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
118672366  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
118677495  proteasome subunit beta type-7-like [KO:K02739] [EC:3.4.25.1]
118667070  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
118672116  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
118675960  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
118679231  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
118659722  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
118673515  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
118666031  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
118659186  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
118674451  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
118655396  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
118665053  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
118671610  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
118676255  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
118662263  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
118671666  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
118678430  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
118649948  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 12-like [KO:K03035]
118671823  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
118671770  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
118667921  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
118674324  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
118656347  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 7-like [KO:K03038]
118648967  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
118673745  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
118662250  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
118674093  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
118663710  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
118665674  SEM1; LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
118654215  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
118675854  TAR DNA-binding protein 43 isoform X1 [KO:K23600]
118656085  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
118650318  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
118650945  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
118659071  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
118661196  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
118654257  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
118676399  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
118676423  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
118676441  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
118666106  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
118676660  heterogeneous nuclear ribonucleoproteins A2/B1-like isoform X1 [KO:K13158]
118678322  ATXN2; ataxin-2 isoform X1 [KO:K23625]
118652882  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
118657838  MATR3; matrin-3 isoform X1 [KO:K13213]
118649094  LOW QUALITY PROTEIN: angiogenin-like [KO:K16631] [EC:3.1.27.-]
118667279  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
118649944  THO complex subunit 4-like [KO:K12881]
118649945  THO complex subunit 4-like [KO:K12881]
118649951  THO complex subunit 4-like [KO:K12881]
118667190  LOW QUALITY PROTEIN: THO complex subunit 4-like [KO:K12881]
118671810  ALYREF; THO complex subunit 4 [KO:K12881]
118657486  THO complex subunit 4-like [KO:K12881]
118657487  THO complex subunit 4-like [KO:K12881]
118651555  serine/arginine-rich splicing factor 3-like [KO:K12892]
118660016  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
118669446  SRSF7; serine/arginine-rich splicing factor 7 [KO:K12896]
118670239  serine/arginine-rich splicing factor 7-like [KO:K12896]
118657461  serine/arginine-rich splicing factor 7-like isoform X1 [KO:K12896]
118649015  NXF1; nuclear RNA export factor 1 isoform X1 [KO:K14284]
118653413  nuclear RNA export factor 2-like [KO:K14284]
118653414  NXF3; LOW QUALITY PROTEIN: nuclear RNA export factor 3 [KO:K14284]
118653416  nuclear RNA export factor 2-like [KO:K14284]
118653418  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
118653419  nuclear RNA export factor 2-like [KO:K14284]
118654141  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
118663506  NXT1; NTF2-related export protein 1 [KO:K14285]
118663512  NTF2-related export protein 1-like [KO:K14285]
118667001  GLE1; nucleoporin GLE1 isoform X1 [KO:K18723]
118673090  TPR; nucleoprotein TPR isoform X1 [KO:K09291]
118650178  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
118677694  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
118664715  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
118663114  RAE1; mRNA export factor isoform X1 [KO:K14298]
118668058  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
118662575  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
118678855  NUP133; nuclear pore complex protein Nup133 isoform X1 [KO:K14300]
118650988  NUP107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
118650900  NUP37; nucleoporin Nup37 isoform X1 [KO:K14302]
118666523  nucleoporin Nup37-like [KO:K14302]
118665037  NUP160; nuclear pore complex protein Nup160 isoform X1 [KO:K14303]
118671155  NUP85; nuclear pore complex protein Nup85 [KO:K14304]
118660805  NUP43; nucleoporin Nup43 isoform X1 [KO:K14305]
118654623  NUP62CL; nucleoporin-62 C-terminal-like protein [KO:K14306]
118655361  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
118650038  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
118670063  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
118674124  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
118666366  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
118666982  NUP188; nucleoporin NUP188 homolog isoform X1 [KO:K14311]
118656084  NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
118661762  NUP35; nucleoporin NUP35 isoform X1 [KO:K14313]
118672902  NUP210L; nuclear pore membrane glycoprotein 210-like isoform X1 [KO:K14314]
118675944  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
118676190  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
118665808  LOW QUALITY PROTEIN: nuclear envelope pore membrane protein POM 121C-like [KO:K14316]
118673714  POM121L2; POM121-like protein 2 [KO:K14316]
118655784  POM121C; nuclear envelope pore membrane protein POM 121C isoform X1 [KO:K14316]
118666945  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
118671966  NUP88; nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
118676720  RANBP2; E3 SUMO-protein ligase RanBP2 isoform X1 [KO:K12172] [EC:2.3.2.-]
118666934  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
118678529  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
118667058  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
118662737  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
118672678  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
118649620  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
118671592  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118678214  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
118655263  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
118659758  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
118664384  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
118675238  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
118656765  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 isoform X1 [KO:K03237]
118649462  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
118666845  cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
118679657  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
118667369  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
118679652  tumor necrosis factor-like isoform X1 [KO:K03156]
118649937  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
118676259  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
118679513  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
118662418  dual specificity mitogen-activated protein kinase kinase 3-like [KO:K04432] [EC:2.7.12.2]
118672023  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
118660246  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
118660247  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
118657384  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
118678505  NOS1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
118665249  CCS; copper chaperone for superoxide dismutase [KO:K04569]
118658304  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
118659650  epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
118668481  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
118675203  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
118656502  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
118656959  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
118665148  CAT; catalase isoform X1 [KO:K03781] [EC:1.11.1.6]
118651724  PRPH; peripherin isoform X1 [KO:K07607]
118659211  NEFL; neurofilament light polypeptide [KO:K04572]
118659329  NEFM; neurofilament medium polypeptide isoform X1 [KO:K04573]
118678250  NEFH; neurofilament heavy polypeptide [KO:K04574]
118669014  PFN4; profilin-4 [KO:K05759]
118671957  PFN1; profilin-1 [KO:K05759]
118657683  PFN3; profilin-3 [KO:K05759]
118659361  ACTB; actin, cytoplasmic 1 [KO:K05692]
118674776  LOW QUALITY PROTEIN: actin-like [KO:K05692]
118671408  ACTG1; actin, cytoplasmic 2 [KO:K05692]
118670145  ANXA7; annexin A7 isoform X1 [KO:K17095]
118670194  ANXA11; annexin A11 [KO:K17095]
118650010  KIF5A; kinesin heavy chain [KO:K10396]
118661865  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
118676723  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
118674327  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
118660065  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
118655023  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
118665266  KLC2; kinesin light chain 2 [KO:K10407]
118650258  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
118650856  tubulin alpha-1B chain-like [KO:K07374]
118666216  tubulin alpha-1C chain-like [KO:K07374]
118673521  tubulin alpha-1B chain-like [KO:K07374]
118678375  LOW QUALITY PROTEIN: putative tubulin-like protein alpha-4B [KO:K07374]
118678448  LOW QUALITY PROTEIN: tubulin alpha-3 chain-like [KO:K07374]
118652035  tubulin alpha-1A chain [KO:K07374]
118661080  TUBA4A; tubulin alpha-4A chain [KO:K07374]
118661627  tubulin alpha-1D chain isoform X1 [KO:K07374]
118672614  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
118658764  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
118679650  TUBB; tubulin beta chain [KO:K07375]
118652991  TUBB3; tubulin beta-3 chain [KO:K07375]
118655285  TUBB4B; tubulin beta-4B chain [KO:K07375]
118662962  TUBB1; tubulin beta-1 chain [KO:K07375]
118663023  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
118675862  tubulin beta-2B chain isoform X1 [KO:K07375]
118676275  TUBB2B; tubulin beta-2B chain [KO:K07375]
118668831  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
118649618  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
118667370  DCTN3; dynactin subunit 3 [KO:K10425]
118658062  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
118656362  dynactin subunit 5-like [KO:K10427]
118656415  DCTN5; dynactin subunit 5 [KO:K10427]
118650968  DCTN6; dynactin subunit 6 isoform X1 [KO:K10428]
118652468  ACTR1B; beta-centractin isoform X1 [KO:K16575]
118669799  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
118655966  ACTR10; actin-related protein 10 [KO:K16576]
118666136  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
118678956  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
118667959  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
118660645  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
118668589  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
118661241  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
118669351  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
118655492  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
118671176  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
118655944  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
118656373  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
118672094  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
118672427  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
118675844  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
118667712  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
118672215  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
118657832  DNAL1; dynein light chain 1, axonemal [KO:K10411]
118650153  DNAL4; dynein light chain 4, axonemal [KO:K10412]
118665137  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
118672123  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
118658054  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
118659034  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
118655320  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
118656558  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
118652139  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
118672228  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
118657505  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
118652122  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
118658935  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
118670346  NRG3; pro-neuregulin-3, membrane-bound isoform [KO:K05457]
118661478  ERBB4; receptor tyrosine-protein kinase erbB-4 [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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Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
  Journal
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x
Reference
PMID: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.
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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
mmyo00190  Oxidative phosphorylation
mmyo03013  Nucleocytoplasmic transport
mmyo03050  Proteasome
mmyo04010  MAPK signaling pathway
mmyo04012  ErbB signaling pathway
mmyo04020  Calcium signaling pathway
mmyo04115  p53 signaling pathway
mmyo04137  Mitophagy - animal
mmyo04140  Autophagy - animal
mmyo04141  Protein processing in endoplasmic reticulum
mmyo04210  Apoptosis
mmyo04724  Glutamatergic synapse
mmyo04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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