KEGG   PATHWAY: elk05014
Entry
elk05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Enhydra lutris kenyoni (northern sea otter)
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
elk05014  Amyotrophic lateral sclerosis
elk05014

Organism
Enhydra lutris kenyoni (northern sea otter) [GN:elk]
Gene
111161133  cellular tumor antigen p53 [KO:K04451]
111159768  BH3-interacting domain death agonist [KO:K04726]
111154217  apoptosis regulator Bcl-2 [KO:K02161]
111141775  bcl-2-like protein 1 [KO:K04570]
111160657  apoptosis regulator BAX isoform X1 [KO:K02159]
111149975  bcl2-associated agonist of cell death [KO:K02158]
111142091  apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
111147305  cytochrome c isoform X1 [KO:K08738]
111142635  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
111141900  LOW QUALITY PROTEIN: uncharacterized protein LOC111141900 [KO:K08738]
111154673  caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
111144607  caspase-3 [KO:K02187] [EC:3.4.22.56]
111155698  superoxide dismutase [Cu-Zn] isoform X1 [KO:K04565] [EC:1.15.1.1]
111149612  NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
111142406  NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
111155738  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
111157253  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
111140937  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111139570  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111152555  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
111147374  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111160211  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
111154239  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
111151938  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
111155596  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
111157547  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
111161756  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
111145557  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
111159811  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
111161328  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
111162309  acyl carrier protein, mitochondrial [KO:K03955]
111161769  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
111142148  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
111143867  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
111148723  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111151936  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
111141342  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
111148802  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 isoform X1 [KO:K03960]
111147306  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
111146284  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
111162542  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
111145242  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
111146716  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
111156034  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
111150594  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
111141377  NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
111138869  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
111149396  NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
111141116  NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
111142993  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
111153450  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
111161695  NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
111149681  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
111149131  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
111154340  NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
111153400  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
111154638  succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
111138865  succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
111153893  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
111139384  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
111146626  cytochrome c1, heme protein, mitochondrial [KO:K00413]
111143869  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
111162256  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
111142816  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
111146514  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
111141027  cytochrome b-c1 complex subunit 8 isoform X1 [KO:K00418]
111160042  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
111161710  cytochrome b-c1 complex subunit 10 [KO:K00420]
111153043  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
111141707  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
111153215  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
111155858  cytochrome c oxidase subunit 5B, mitochondrial isoform X1 [KO:K02265]
111162190  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
111156401  cytochrome c oxidase subunit 6A1, mitochondrial isoform X1 [KO:K02266]
111161463  cytochrome c oxidase subunit 6B1 isoform X1 [KO:K02267]
111155471  cytochrome c oxidase subunit 6B1-like [KO:K02267]
111139590  cytochrome c oxidase subunit 6B2 [KO:K02267]
111147664  cytochrome c oxidase subunit 6C [KO:K02268]
111146812  cytochrome c oxidase subunit 6C [KO:K02268]
111161502  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
111147649  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
111158437  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
111146524  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111156868  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
111160926  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111149314  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
111149315  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
111148335  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111152225  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111160948  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
111149630  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
111152453  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
111142536  ATP synthase subunit alpha, mitochondrial isoform X1 [KO:K02132]
111160107  ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
111158608  ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
111161765  ATP synthase subunit delta, mitochondrial [KO:K02134]
111152423  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
111141583  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
111143342  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
111150035  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
111157857  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
111151344  ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
111160337  ATP synthase subunit d, mitochondrial [KO:K02138]
111155772  ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
111147126  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
111151840  ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
111141207  voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
111160709  mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
111138901  mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518]
111145292  calcium uniporter protein, mitochondrial [KO:K20858]
111154572  serine/threonine-protein kinase PINK1, mitochondrial [KO:K05688] [EC:2.7.11.1]
111158711  E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
111155530  optineurin isoform X1 [KO:K19946]
111158853  sequestosome-1 [KO:K14381]
111150637  microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
111152982  microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
111141551  microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
111142106  serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
111152126  TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
111148360  WD repeat-containing protein 41 [KO:K23610]
111139265  guanine nucleotide exchange protein SMCR8 [KO:K23611]
111159865  guanine nucleotide exchange C9orf72 homolog [KO:K23609]
111144096  ras-related protein Rab-8A [KO:K07901]
111139209  ras-related protein Rab-39B [KO:K07925]
111148163  ras-related protein Rab-1A [KO:K07874]
111154883  serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
111140006  serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
111139478  serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
111158002  autophagy-related protein 101 isoform X1 [KO:K19730]
111149764  autophagy-related protein 13 isoform X1 [KO:K08331]
111155388  RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
111149427  activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
111150658  beclin-2 [KO:K08334]
111151520  beclin-1 isoform X1 [KO:K08334]
111155119  phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
111162436  beclin 1-associated autophagy-related key regulator [KO:K17889]
111144899  nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
111142649  phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
111149620  autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
111148489  autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
111145977  WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
111158171  WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
111143863  alsin isoform X1 [KO:K04575]
111155079  ras-related protein Rab-5A [KO:K07887]
111145636  ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
111148914  charged multivesicular body protein 2b [KO:K12192]
111158261  polyphosphoinositide phosphatase [KO:K22913] [EC:3.1.3.-]
111140645  spatacsin isoform X1 [KO:K19026]
111144797  serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111154144  serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111154566  serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111148007  calcineurin subunit B type 1 [KO:K06268]
111146053  calcineurin subunit B type 2 [KO:K06268]
111139788  ubiquilin-2 isoform X1 [KO:K04523]
111159601  ubiquilin-3 [KO:K04523]
111138849  ubiquilin-4 isoform X1 [KO:K04523]
111153560  ubiquilin-1 isoform X1 [KO:K04523]
111145449  transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
111146592  derlin-1 isoform X1 [KO:K11519]
111161782  proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
111157026  proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
111152671  proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
111142650  LOW QUALITY PROTEIN: proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
111141333  proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
111143461  proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
111159417  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
111140663  proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
111161100  proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
111146069  proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
111151691  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
111143148  proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
111139193  proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
111158705  proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
111161949  proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
111162024  proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
111155299  26S proteasome regulatory subunit 7 [KO:K03061]
111148465  26S proteasome regulatory subunit 4 [KO:K03062]
111161514  26S proteasome regulatory subunit 6B isoform X1 [KO:K03063]
111139115  26S proteasome regulatory subunit 10B [KO:K03064]
111149577  26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
111158036  26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
111148031  26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
111143971  26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
111151573  26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
111156408  26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
111158181  26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
111151359  26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
111144078  26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
111152968  26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
111149610  26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
111161952  26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
111140976  26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
111161550  26S proteasome non-ATPase regulatory subunit 8 isoform X1 [KO:K03031]
111141807  LOW QUALITY PROTEIN: proteasomal ubiquitin receptor ADRM1 [KO:K06691]
111147630  LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
111150520  histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
111154646  TAR DNA-binding protein 43 isoform X1 [KO:K23600]
111154719  TAR DNA-binding protein 43 isoform X1 [KO:K23600]
111162225  RNA-binding protein FUS isoform X1 [KO:K13098]
111155424  heterogeneous nuclear ribonucleoprotein A1-like isoform X1 [KO:K12741]
111159993  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
111157124  heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
111146599  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111144503  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111154429  heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
111157903  heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
111143104  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111147225  heterogeneous nuclear ribonucleoproteins A2/B1 [KO:K13158]
111156347  ataxin-2 isoform X1 [KO:K23625]
111162230  LOW QUALITY PROTEIN: ataxin-2-like protein [KO:K23625]
111141125  matrin-3 isoform X1 [KO:K13213]
111161137  angiogenin [KO:K16631] [EC:3.1.27.-]
111145548  nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
111160380  THO complex subunit 4 [KO:K12881]
111147622  serine/arginine-rich splicing factor 3 [KO:K12892]
111158415  serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
111150211  nuclear RNA export factor 1 [KO:K14284]
111150213  nuclear RNA export factor 1 [KO:K14284]
111139886  LOW QUALITY PROTEIN: nuclear RNA export factor 5-like [KO:K14284]
111139891  nuclear RNA export factor 3 [KO:K14284]
111139896  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
111139897  nuclear RNA export factor 2-like [KO:K14284]
111160960  NTF2-related export protein 2 isoform X1 [KO:K14285]
111141485  NTF2-related export protein 1 [KO:K14285]
111145835  nucleoporin GLE1 isoform X1 [KO:K18723]
111150655  nucleoprotein TPR isoform X1 [KO:K09291]
111157573  nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
111156648  nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
111159374  nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
111141667  mRNA export factor [KO:K14298]
111143848  protein SEC13 homolog [KO:K14004]
111142644  nucleoporin SEH1 isoform X1 [KO:K14299]
111160511  nuclear pore complex protein Nup133 [KO:K14300]
111142103  nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
111142195  nucleoporin Nup37 isoform X1 [KO:K14302]
111160269  nuclear pore complex protein Nup85 [KO:K14304]
111147623  nucleoporin Nup43 [KO:K14305]
111160654  nuclear pore glycoprotein p62 [KO:K14306]
111161315  nucleoporin-62 C-terminal-like protein [KO:K14306]
111159904  nucleoporin p58/p45 isoform X1 [KO:K14307]
111149064  nucleoporin p54 isoform X1 [KO:K14308]
111157322  nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
111151974  nuclear pore complex protein Nup205 [KO:K14310]
111145656  nucleoporin NUP188 homolog [KO:K14311]
111141065  nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
111141660  nucleoporin NUP53 isoform X1 [KO:K14313]
111159061  nuclear pore membrane glycoprotein 210 [KO:K14314]
111138966  nuclear pore membrane glycoprotein 210-like isoform X1 [KO:K14314]
111143425  nucleoporin NDC1 isoform X1 [KO:K14315]
111146430  LOW QUALITY PROTEIN: nuclear envelope pore membrane protein POM 121C-like [KO:K14316]
111162576  POM121-like protein 2 [KO:K14316]
111145520  nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
111161109  nuclear pore complex protein Nup88 [KO:K14318]
111155833  LOW QUALITY PROTEIN: E3 SUMO-protein ligase RanBP2-like [KO:K12172] [EC:2.3.2.-]
111146140  probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.-]
111142584  coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial isoform X1 [KO:K22759]
111146005  78 kDa glucose-regulated protein isoform X1 [KO:K09490] [EC:3.6.4.10]
111141408  vesicle-associated membrane protein-associated protein B/C [KO:K10707]
111151136  cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
111160240  DNA damage-inducible transcript 3 protein [KO:K04452]
111158091  serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
111160084  LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
111145476  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
111146100  TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
111147502  mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
111153889  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
111148064  eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
111148773  eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
111157635  cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
111152507  inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
111145834  sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
111152248  tumor necrosis factor isoform X1 [KO:K03156]
111159748  tumor necrosis factor receptor superfamily member 1A [KO:K03158]
111154868  tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
111152240  death domain-associated protein 6 isoform X1 [KO:K02308]
111139240  dual specificity mitogen-activated protein kinase kinase 3 [KO:K04432] [EC:2.7.12.2]
111158041  dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
111152267  mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
111157625  mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
111157628  mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
111152183  mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
111156411  nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
111150046  copper chaperone for superoxide dismutase isoform X1 [KO:K04569]
111156930  probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
111158122  glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
111162615  glutathione peroxidase 6-like [KO:K00432] [EC:1.11.1.9]
111143439  glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
111143890  glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
111162616  epididymal secretory glutathione peroxidase isoform X2 [KO:K00432] [EC:1.11.1.9]
111140869  glutathione peroxidase 3 isoform X1 [KO:K00432] [EC:1.11.1.9]
111149443  catalase isoform X1 [KO:K03781] [EC:1.11.1.6]
111157920  peripherin [KO:K07607]
111155116  neurofilament light polypeptide [KO:K04572]
111156545  neurofilament medium polypeptide [KO:K04573]
111160049  LOW QUALITY PROTEIN: neurofilament heavy polypeptide [KO:K04574]
111161067  profilin-1 [KO:K05759]
111139390  profilin-2-like [KO:K05759]
111154122  profilin-4 [KO:K05759]
111141302  profilin-3 [KO:K05759]
111151499  profilin-2 isoform X1 [KO:K05759]
111160249  actin, cytoplasmic 2 [KO:K05692]
111145734  actin, cytoplasmic 1 [KO:K05692]
111144864  annexin A7 isoform X1 [KO:K17095]
111144959  annexin A11 [KO:K17095]
111150076  LOW QUALITY PROTEIN: annexin A7-like [KO:K17095]
111155551  kinesin-1 heavy chain [KO:K10396]
111160138  kinesin heavy chain isoform X1 [KO:K10396]
111142050  kinesin heavy chain [KO:K10396]
111160692  kinesin light chain 3 isoform X1 [KO:K10407]
111148551  kinesin light chain 1 isoform X1 [KO:K10407]
111149675  kinesin light chain 2 isoform X1 [KO:K10407]
111147688  kinesin light chain 4 isoform X1 [KO:K10407]
111143142  tubulin alpha-4A chain [KO:K07374]
111142067  tubulin alpha-1A chain-like [KO:K07374]
111157712  tubulin alpha-1B chain [KO:K07374]
111157713  tubulin alpha-1A chain [KO:K07374]
111158883  tubulin alpha-1A chain-like [KO:K07374]
111159721  tubulin alpha-8 chain isoform X1 [KO:K07374]
111157791  tubulin alpha-1C chain [KO:K07374]
111144980  putative tubulin-like protein alpha-4B [KO:K07374]
111151071  tubulin alpha-8 chain isoform X1 [KO:K07374]
111158635  LOW QUALITY PROTEIN: tubulin alpha chain-like 3 [KO:K07374]
111142377  tubulin alpha-3 chain [KO:K07374]
111142381  tubulin alpha-3 chain [KO:K07374]
111141694  tubulin beta-1 chain [KO:K07375]
111142513  tubulin beta-6 chain isoform X1 [KO:K07375]
111161751  tubulin beta-4A chain isoform X1 [KO:K07375]
111156662  tubulin beta-2A chain isoform X1 [KO:K07375]
111152799  tubulin beta-3 chain [KO:K07375]
111156744  tubulin beta-2B chain isoform X1 [KO:K07375]
111146420  tubulin beta-4B chain isoform X1 [KO:K07375]
111152509  tubulin beta chain isoform X1 [KO:K07375]
111147910  dynactin subunit 1 isoform X1 [KO:K04648]
111160098  dynactin subunit 2 isoform X1 [KO:K10424]
111145732  dynactin subunit 3 isoform X1 [KO:K10425]
111141168  dynactin subunit 4 isoform X1 [KO:K10426]
111162315  dynactin subunit 5 [KO:K10427]
111144608  dynactin subunit 6 [KO:K10428]
111144862  alpha-centractin [KO:K16575]
111155921  beta-centractin [KO:K16575]
111160254  actin-related protein 10 [KO:K16576]
111151488  LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
111147958  dynein heavy chain 6, axonemal [KO:K10408]
111147239  dynein heavy chain 11, axonemal [KO:K10408]
111150822  dynein heavy chain 14, axonemal [KO:K10408]
111161130  dynein heavy chain 9, axonemal [KO:K10408]
111161163  dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
111143860  dynein heavy chain 12, axonemal [KO:K10408]
111145753  dynein heavy chain 3, axonemal [KO:K10408]
111156345  LOW QUALITY PROTEIN: dynein heavy chain 10, axonemal-like [KO:K10408]
111143889  dynein heavy chain 1, axonemal [KO:K10408]
111160287  dynein heavy chain 17, axonemal [KO:K10408]
111156405  dynein heavy chain 7, axonemal [KO:K10408]
111147554  dynein heavy chain 8, axonemal [KO:K10408]
111153413  dynein heavy chain 5, axonemal [KO:K10408]
111153412  dynein heavy chain 5, axonemal-like [KO:K10408]
111143650  LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
111145979  dynein intermediate chain 1, axonemal [KO:K10409]
111158169  dynein intermediate chain 2, axonemal [KO:K11143]
111148699  dynein light chain 1, axonemal [KO:K10411]
111157606  dynein light chain 4, axonemal isoform X1 [KO:K10412]
111149732  excitatory amino acid transporter 2 [KO:K05613]
111151537  nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
111140968  glutamate receptor 1 isoform X1 [KO:K05197]
111153762  glutamate receptor 2 isoform X1 [KO:K05198]
111145480  glutamate receptor ionotropic, NMDA 1-like [KO:K05208]
111145775  glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
111156070  glutamate receptor ionotropic, NMDA 2A [KO:K05209]
111142166  glutamate receptor ionotropic, NMDA 2B [KO:K05210]
111160373  glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
111160688  glutamate receptor ionotropic, NMDA 2D [KO:K05212]
111144494  pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
111141189  pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
111145254  pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
111138855  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
Reference
PMID:28700839
  Authors
Brown RH, Al-Chalabi A
  Title
Amyotrophic Lateral Sclerosis.
  Journal
N Engl J Med 377:162-172 (2017)
DOI:10.1056/NEJMra1603471
Reference
PMID:28512398
  Authors
Webster CP, Smith EF, Shaw PJ, De Vos KJ
  Title
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
  Journal
Front Mol Neurosci 10:123 (2017)
DOI:10.3389/fnmol.2017.00123
Reference
PMID:30879475
  Authors
Valko K, Ciesla L
  Title
Amyotrophic lateral sclerosis.
  Journal
Prog Med Chem 58:63-117 (2019)
DOI:10.1016/bs.pmch.2018.12.001
Reference
PMID:30870681
  Authors
Mathis S, Goizet C, Soulages A, Vallat JM, Masson GL
  Title
Genetics of amyotrophic lateral sclerosis: A review.
  Journal
J Neurol Sci 399:217-226 (2019)
DOI:10.1016/j.jns.2019.02.030
Reference
PMID:23415570
  Authors
Turner MR, Hardiman O, Benatar M, Brooks BR, Chio A, de Carvalho M, Ince PG, Lin C, Miller RG, Mitsumoto H, Nicholson G, Ravits J, Shaw PJ, Swash M, Talbot K, Traynor BJ, Van den Berg LH, Veldink JH, Vucic S, Kiernan MC
  Title
Controversies and priorities in amyotrophic lateral sclerosis.
  Journal
Lancet Neurol 12:310-22 (2013)
DOI:10.1016/S1474-4422(13)70036-X
Reference
PMID:16924260
  Authors
Pasinelli P, Brown RH
  Title
Molecular biology of amyotrophic lateral sclerosis: insights from genetics.
  Journal
Nat Rev Neurosci 7:710-23 (2006)
DOI:10.1038/nrn1971
Reference
PMID:16723044
  Authors
Goodall EF, Morrison KE
  Title
Amyotrophic lateral sclerosis (motor neuron disease): proposed mechanisms and pathways to treatment.
  Journal
Expert Rev Mol Med 8:1-22 (2006)
DOI:10.1017/S1462399406010854
Reference
PMID:28669745
  Authors
Smith EF, Shaw PJ, De Vos KJ
  Title
The role of mitochondria in amyotrophic lateral sclerosis.
  Journal
Neurosci Lett 710:132933 (2019)
DOI:10.1016/j.neulet.2017.06.052
Reference
PMID:32344665
  Authors
Kodavati M, Wang H, Hegde ML
  Title
Altered Mitochondrial Dynamics in Motor Neuron Disease: An Emerging Perspective.
  Journal
Cells 9:E1065 (2020)
DOI:10.3390/cells9041065
Reference
PMID:16372325
  Authors
Hervias I, Beal MF, Manfredi G
  Title
Mitochondrial dysfunction and amyotrophic lateral sclerosis.
  Journal
Muscle Nerve 33:598-608 (2006)
DOI:10.1002/mus.20489
Reference
PMID:16603792
  Authors
Bacman SR, Bradley WG, Moraes CT
  Title
Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target?
  Journal
Mol Neurobiol 33:113-31 (2006)
DOI:10.1385/MN:33:2:113
Reference
PMID:17051205
  Authors
Lin MT, Beal MF
  Title
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.
  Journal
Nature 443:787-95 (2006)
DOI:10.1038/nature05292
Reference
PMID:16909017
  Authors
Bendotti C, Bao Cutrona M, Cheroni C, Grignaschi G, Lo Coco D, Peviani M, Tortarolo M, Veglianese P, Zennaro E
  Title
Inter- and intracellular signaling in amyotrophic lateral sclerosis: role of p38 mitogen-activated protein kinase.
  Journal
Neurodegener Dis 2:128-34 (2005)
DOI:10.1159/000089617
Reference
PMID:14569202
  Authors
Simpson EP, Yen AA, Appel SH
  Title
Oxidative Stress: a common denominator in the pathogenesis of amyotrophic lateral sclerosis.
  Journal
Curr Opin Rheumatol 15:730-6 (2003)
DOI:10.1097/00002281-200311000-00008
Reference
PMID:15310460
  Authors
Bendotti C, Carri MT
  Title
Lessons from models of SOD1-linked familial ALS.
  Journal
Trends Mol Med 10:393-400 (2004)
DOI:10.1016/j.molmed.2004.06.009
Reference
PMID:12595144
  Authors
Robertson J, Kriz J, Nguyen MD, Julien JP
  Title
Pathways to motor neuron degeneration in transgenic mouse models.
  Journal
Biochimie 84:1151-60 (2002)
DOI:10.1016/S0300-9084(02)00025-1
Reference
PMID:17434459
  Authors
Eve DJ, Dennis JS, Citron BA
  Title
Transcription factor p53 in degenerating spinal cords.
  Journal
Brain Res 1150:174-81 (2007)
DOI:10.1016/j.brainres.2007.02.088
Reference
PMID:27649160
  Authors
Bahar E, Kim H, Yoon H
  Title
ER Stress-Mediated Signaling: Action Potential and Ca(2+) as Key Players.
  Journal
Int J Mol Sci 17:E1558 (2016)
DOI:10.3390/ijms17091558
Reference
PMID:19230774
  Authors
Bezprozvanny I
  Title
Calcium signaling and neurodegenerative diseases.
  Journal
Trends Mol Med 15:89-100 (2009)
DOI:10.1016/j.molmed.2009.01.001
Reference
PMID:16026864
  Authors
von Lewinski F, Keller BU
  Title
Ca2+, mitochondria and selective motoneuron vulnerability: implications for ALS.
  Journal
Trends Neurosci 28:494-500 (2005)
DOI:10.1016/j.tins.2005.07.001
Reference
PMID:11239414
  Authors
Julien JP.
  Title
Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded.
  Journal
Cell 104:581-91 (2001)
DOI:10.1016/S0092-8674(01)00244-6
Reference
PMID:11870681
  Authors
Hand CK, Rouleau GA
  Title
Familial amyotrophic lateral sclerosis.
  Journal
Muscle Nerve 25:135-59 (2002)
DOI:10.1002/mus.10000
Reference
PMID:16713195
  Authors
Barber SC, Mead RJ, Shaw PJ
  Title
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic target.
  Journal
Biochim Biophys Acta 1762:1051-67 (2006)
DOI:10.1016/j.bbadis.2006.03.008
Reference
PMID:30837838
  Authors
Prasad A, Bharathi V, Sivalingam V, Girdhar A, Patel BK
  Title
Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis.
  Journal
Front Mol Neurosci 12:25 (2019)
DOI:10.3389/fnmol.2019.00025
Reference
PMID:29626651
  Authors
Nguyen DKH, Thombre R, Wang J
  Title
Autophagy as a common pathway in amyotrophic lateral sclerosis.
  Journal
Neurosci Lett 697:34-48 (2019)
DOI:10.1016/j.neulet.2018.04.006
Reference
PMID:26264610
  Authors
Lee JK, Shin JH, Lee JE, Choi EJ
  Title
Role of autophagy in the pathogenesis of amyotrophic lateral sclerosis.
  Journal
Biochim Biophys Acta 1852:2517-24 (2015)
DOI:10.1016/j.bbadis.2015.08.005
Reference
PMID:28148298
  Authors
Oakes JA, Davies MC, Collins MO
  Title
TBK1: a new player in ALS linking autophagy and neuroinflammation.
  Journal
Mol Brain 10:5 (2017)
DOI:10.1186/s13041-017-0287-x
Reference
PMID:28878620
  Authors
Ramesh N, Pandey UB
  Title
Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.
  Journal
Front Mol Neurosci 10:263 (2017)
DOI:10.3389/fnmol.2017.00263
Reference
PMID:17409386
  Authors
Kunita R, Otomo A, Mizumura H, Suzuki-Utsunomiya K, Hadano S, Ikeda JE
  Title
The Rab5 activator ALS2/alsin acts as a novel Rac1 effector through Rac1-activated endocytosis.
  Journal
J Biol Chem 282:16599-611 (2007)
DOI:10.1074/jbc.M610682200
Reference
PMID:17566607
  Authors
Hadano S, Kunita R, Otomo A, Suzuki-Utsunomiya K, Ikeda JE
  Title
Molecular and cellular function of ALS2/alsin: implication of membrane dynamics in neuronal development and degeneration.
  Journal
Neurochem Int 51:74-84 (2007)
DOI:10.1016/j.neuint.2007.04.010
Reference
PMID:26388731
  Authors
Ruffoli R, Bartalucci A, Frati A, Fornai F
  Title
Ultrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesis.
  Journal
Front Cell Neurosci 9:341 (2015)
DOI:10.3389/fncel.2015.00341
Reference
PMID:25991442
  Authors
Menzies FM, Fleming A, Rubinsztein DC
  Title
Compromised autophagy and neurodegenerative diseases.
  Journal
Nat Rev Neurosci 16:345-57 (2015)
DOI:10.1038/nrn3961
Reference
PMID:32119873
  Authors
Casterton RL, Hunt RJ, Fanto M
  Title
Pathomechanism Heterogeneity in the Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disease Spectrum: Providing Focus Through the Lens of Autophagy.
  Journal
J Mol Biol 432:2692-2713 (2020)
DOI:10.1016/j.jmb.2020.02.018
Reference
PMID:28057298
  Authors
Lin G, Mao D, Bellen HJ
  Title
Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload.
  Journal
Curr Top Dev Biol 121:111-171 (2017)
DOI:10.1016/bs.ctdb.2016.07.004
Reference
PMID:24619348
  Authors
Hetz C, Mollereau B
  Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
  Journal
Nat Rev Neurosci 15:233-49 (2014)
DOI:10.1038/nrn3689
Reference
PMID:27815720
  Authors
Xiang C, Wang Y, Zhang H, Han F
  Title
The role of endoplasmic reticulum stress in neurodegenerative disease.
  Journal
Apoptosis 22:1-26 (2017)
DOI:10.1007/s10495-016-1296-4
Reference
PMID:29476642
  Authors
Hughes D, Mallucci GR
  Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
  Journal
FEBS J 286:342-355 (2019)
DOI:10.1111/febs.14422
Reference
PMID:21722302
  Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
  Title
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.
  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
elk00190  Oxidative phosphorylation
elk03013  Nucleocytoplasmic transport
elk03050  Proteasome
elk04010  MAPK signaling pathway
elk04012  ErbB signaling pathway
elk04020  Calcium signaling pathway
elk04115  p53 signaling pathway
elk04137  Mitophagy - animal
elk04140  Autophagy - animal
elk04141  Protein processing in endoplasmic reticulum
elk04210  Apoptosis
elk04724  Glutamatergic synapse
elk04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

DBGET integrated database retrieval system