KEGG   PATHWAY: caty05014
Entry
caty05014                   Pathway                                
Name
Amyotrophic lateral sclerosis - Cercocebus atys (sooty mangabey)
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
caty05014  Amyotrophic lateral sclerosis
caty05014

Organism
Cercocebus atys (sooty mangabey) [GN:caty]
Gene
105582225  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
105588753  caspase-1-like [KO:K01370] [EC:3.4.22.36]
105588757  caspase-1-like isoform X1 [KO:K01370] [EC:3.4.22.36]
105591256  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
105590006  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
105582236  BCL2L1; bcl-2-like protein 1 isoform X1 [KO:K04570]
105595265  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
105577493  BAD; bcl2-associated agonist of cell death [KO:K02158]
105573341  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
105597193  uncharacterized protein LOC105597193 [KO:K08738]
105571593  cytochrome c-like [KO:K08738]
105588083  cytochrome c [KO:K08738]
105595720  cytochrome c-like [KO:K08738]
105589984  cytochrome c-like [KO:K08738]
105590205  cytochrome c-like [KO:K08738]
105599419  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
105593734  LOW QUALITY PROTEIN: caspase-3-like [KO:K02187] [EC:3.4.22.56]
105597738  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
105575354  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
26408639  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
26408637  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
26408647  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
26408626  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
26408654  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
26408642  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
26408645  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
105577340  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
105579316  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
105575491  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
105599894  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105599368  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105595659  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947]
105576651  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
105587266  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
105589594  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
105598134  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
105598509  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
105583580  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
105572148  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
105593821  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
105600033  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
105582997  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
105577713  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
105586149  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
105581958  acyl carrier protein, mitochondrial [KO:K03955]
105584063  acyl carrier protein, mitochondrial-like [KO:K03955]
105591845  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 isoform X1 [KO:K03956]
105573373  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
105601736  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
105596585  NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
105589670  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
105586631  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
105580028  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X1 [KO:K03959]
105589747  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
105597722  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
105590093  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
105601592  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
105581934  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
105598708  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
105573646  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
105590516  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
105580207  NDUFS1; LOW QUALITY PROTEIN: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
105577327  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial-like [KO:K03935] [EC:7.1.1.2]
105589249  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
105592827  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
105598089  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
105594723  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 isoform X1 [KO:K03938]
105585999  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
105591714  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
105577334  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
105580800  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
105578690  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
105600716  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like [KO:K00234] [EC:1.3.5.1]
105576147  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
105597674  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
105576571  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial-like [KO:K00235] [EC:1.3.5.1]
105589241  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
105588694  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
105578094  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
26408652  CYTB; cytochrome b [KO:K00412]
105581237  cytochrome c1, heme protein, mitochondrial [KO:K00413]
105573662  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
105586358  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
105571349  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
105594572  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
105586502  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
105599753  cytochrome b-c1 complex subunit 8 [KO:K00418]
105591726  cytochrome b-c1 complex subunit 10 [KO:K00420]
26408629  COX3; cytochrome c oxidase subunit III [KO:K02262]
26408656  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
26408635  COX2; cytochrome c oxidase subunit II [KO:K02261]
105594544  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
105582233  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
105574517  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
105582641  cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
105600051  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
105589874  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
105583702  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
105590937  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
105599616  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
105578181  cytochrome c oxidase subunit 6B1 [KO:K02267]
105582753  cytochrome c oxidase subunit 6B2 [KO:K02267]
105586528  cytochrome c oxidase subunit 6C [KO:K02268]
105578217  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
105588042  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
105597100  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
105596112  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
105596144  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
105574720  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
105577509  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
105596604  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
105592536  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
105587286  ATP5B; LOW QUALITY PROTEIN: ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
105585296  ATP synthase subunit gamma, mitochondrial [KO:K02136]
105597411  ATP5C1; LOW QUALITY PROTEIN: ATP synthase subunit gamma, mitochondrial [KO:K02136]
105591705  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
105585312  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
26408628  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
105594385  ATP5F1; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
105576012  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
105579472  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
105579834  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
105580260  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
105577747  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
105575580  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
105575934  ATP synthase subunit O, mitochondrial [KO:K02137]
105586569  LOW QUALITY PROTEIN: ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
26408643  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
105599763  VDAC1; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1 [KO:K05862]
105595124  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
105589244  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
105597312  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
105595041  PINK1; serine/threonine-protein kinase PINK1, mitochondrial [KO:K05688] [EC:2.7.11.1]
105594235  PARK2; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
105573814  OPTN; optineurin [KO:K19946]
105590410  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
105592330  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
105582298  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A isoform X1 [KO:K10435]
105574935  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
105600541  TBK1; serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
105576040  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
105599184  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
105598217  SMCR8; smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611]
105591290  protein C9orf72 homolog isoform X1 [KO:K23609]
105571314  RAB8A; ras-related protein Rab-8A [KO:K07901]
105573755  RAB39B; ras-related protein Rab-39B [KO:K07925]
105576478  RAB1A; ras-related protein Rab-1A isoform X1 [KO:K07874]
105580870  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
105591065  ULK1; LOW QUALITY PROTEIN: serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
105593728  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
105580322  ATG101; autophagy-related protein 101 [KO:K19730]
105592799  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
105592129  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
105592798  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
105579690  BECN1; beclin-1 isoform X1 [KO:K08334]
105574934  BECN2; beclin-2 [KO:K08334]
105578339  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
105586652  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
105581494  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
105592559  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
105577472  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
105596658  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
105577812  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
105596945  WD repeat domain phosphoinositide-interacting protein 2-like [KO:K17908]
105598330  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
105580049  ALS2; alsin isoform X1 [KO:K04575]
105578300  RAB5A; ras-related protein Rab-5A [KO:K07887]
105584356  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
105583589  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
105581358  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
105588162  SPG11; spatacsin isoform X1 [KO:K19026]
105571365  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
105579229  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
105597329  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
105593014  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
105576501  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
105578883  UBQLN3; ubiquilin-3 [KO:K04523]
105594500  UBQLN4; ubiquilin-4 [KO:K04523]
105571592  UBQLN2; ubiquilin-2 [KO:K04523]
105584230  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
105598108  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
105598661  DERL1; derlin-1 isoform X1 [KO:K11519]
105589066  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
105596930  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
105571837  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
105592367  PSMA7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
105592635  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
105594335  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
105595723  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
105586681  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
105583495  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
105576297  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
105579529  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
105594775  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
105598784  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
105597518  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
105590230  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
105583181  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
105596561  PSMC1; 26S protease regulatory subunit 4 isoform X1 [KO:K03062]
105597965  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
105586626  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
105592822  PSMC3; 26S protease regulatory subunit 6A isoform X1 [KO:K03065]
105590742  PSMC5; 26S protease regulatory subunit 8 isoform X1 [KO:K03066]
105573973  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
105591601  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
105579563  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
105590975  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
105599325  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
105589388  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
105585651  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
105580298  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 isoform X1 [KO:K03038]
105577178  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
105590303  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
105576041  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
105594561  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
105592361  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
105592205  SHFM1; 26S proteasome complex subunit DSS1 isoform X1 [KO:K10881]
105573127  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
105580876  TARDBP; LOW QUALITY PROTEIN: TAR DNA-binding protein 43 [KO:K23600]
105599559  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
105571580  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
105579858  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
105580240  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
105572731  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105573324  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105573879  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105597636  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105598392  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105583929  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105591315  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
105595704  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
105573187  ATXN2; LOW QUALITY PROTEIN: ataxin-2 [KO:K23625]
105598633  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
105599853  MATR3; LOW QUALITY PROTEIN: matrin-3 [KO:K13213]
105593061  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
105588588  ALYREF; THO complex subunit 4 [KO:K12881]
105592584  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
105599294  serine/arginine-rich splicing factor 3 [KO:K12892]
105597199  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
105577534  NXF1; nuclear RNA export factor 1 isoform X1 [KO:K14284]
105574326  NXF3; nuclear RNA export factor 3 isoform X1 [KO:K14284]
105574335  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
105592070  nuclear RNA export factor 2 [KO:K14284]
105581989  NXT1; NTF2-related export protein 1 [KO:K14285]
105598458  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
105576222  GLE1; nucleoporin GLE1 [KO:K18723]
105579955  TPR; nucleoprotein TPR isoform X1 [KO:K09291]
105586932  NUP50; nuclear pore complex protein Nup50 [KO:K14295]
105572581  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
105578812  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
105585341  RAE1; mRNA export factor [KO:K14298]
105585780  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
105598943  SEH1L; nucleoporin SEH1 [KO:K14299]
105574825  NUP133; nuclear pore complex protein Nup133 isoform X1 [KO:K14300]
105600502  NUP107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
105573308  NUP37; nucleoporin Nup37 isoform X1 [KO:K14302]
105592835  NUP160; nuclear pore complex protein Nup160 isoform X1 [KO:K14303]
105577738  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
105588821  NUP43; nucleoporin Nup43 [KO:K14305]
105572394  NUP62CL; nucleoporin-62 C-terminal-like protein isoform X1 [KO:K14306]
105595315  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
105597031  NUPL1; nucleoporin p58/p45 isoform X1 [KO:K14307]
105576856  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
105588290  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
105589623  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
105576209  NUP188; nucleoporin NUP188 homolog isoform X1 [KO:K14311]
105583523  NUP155; nuclear pore complex protein Nup155 [KO:K14312]
105580186  NUP35; nucleoporin NUP53 [KO:K14313]
105585810  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
105593485  NUP210L; LOW QUALITY PROTEIN: nuclear pore membrane glycoprotein 210-like [KO:K14314]
105596017  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
105593191  POM121L2; POM121-like protein 2 [KO:K14316]
105593367  nuclear envelope pore membrane protein POM 121C [KO:K14316]
105576168  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
105583153  NUP88; nuclear pore complex protein Nup88 [KO:K14318]
105591200  RANBP2; E3 SUMO-protein ligase RanBP2 [KO:K12172] [EC:2.3.2.-]
105594164  SETX; LOW QUALITY PROTEIN: probable helicase senataxin [KO:K10706] [EC:5.6.2.5 5.6.2.3]
105599387  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
105576280  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
105585325  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
105589230  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
105587252  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
105590750  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105575685  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
105580591  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
105600400  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
105588760  caspase-12 [KO:K04741] [EC:3.4.22.-]
105598836  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
105582856  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
105572871  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
105593244  ITPR3; LOW QUALITY PROTEIN: inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
105590050  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
105573465  TNF; tumor necrosis factor [KO:K03156]
105583011  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
105580845  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B [KO:K05141]
105593386  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
105593750  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
105577799  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
105592690  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
105592727  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
105586882  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
105586884  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
105592744  NOS1; LOW QUALITY PROTEIN: nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
105577383  CCS; copper chaperone for superoxide dismutase isoform X1 [KO:K04569]
105593144  GPX5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
105593145  GPX6; LOW QUALITY PROTEIN: glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
105596037  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
105573690  GPX1; LOW QUALITY PROTEIN: glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
105589898  GPX3; LOW QUALITY PROTEIN: glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
105582704  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
105582834  GPX2; LOW QUALITY PROTEIN: glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
105586792  CAT; catalase [KO:K03781] [EC:1.11.1.6]
105580386  PRPH; peripherin isoform X1 [KO:K07607]
105579194  NEFL; neurofilament light polypeptide [KO:K04572]
105579195  NEFM; neurofilament medium polypeptide isoform X1 [KO:K04573]
105575700  NEFH; LOW QUALITY PROTEIN: neurofilament heavy polypeptide [KO:K04574]
105578536  PFN4; profilin-4 [KO:K05759]
105581725  PFN3; profilin-3 [KO:K05759]
105597363  PFN2; profilin-2 isoform X1 [KO:K05759]
105583310  PFN1; profilin-1 [KO:K05759]
105577106  LOW QUALITY PROTEIN: actin, alpha skeletal muscle-like [KO:K05692]
105588610  ACTG1; actin, cytoplasmic 2 [KO:K05692]
105574978  LOW QUALITY PROTEIN: actin-like [KO:K05692]
105598339  ACTB; actin, cytoplasmic 1 [KO:K05692]
105572330  ANXA11; annexin A11 isoform X1 [KO:K17095]
105597327  ANXA7; annexin A7 isoform X1 [KO:K17095]
105587250  KIF5A; kinesin heavy chain isoform 5A [KO:K10396]
105596973  KIF5B; kinesin-1 heavy chain [KO:K10396]
105575961  KIF5C; kinesin heavy chain isoform 5C [KO:K10396]
105577402  KLC2; kinesin light chain 2 [KO:K10407]
105595144  KLC3; LOW QUALITY PROTEIN: kinesin light chain 3 [KO:K10407]
105596749  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
105591521  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
105587029  tubulin alpha-4A chain isoform X1 [KO:K07374]
105571676  tubulin alpha-3 chain isoform X1 [KO:K07374]
105580387  LOW QUALITY PROTEIN: tubulin alpha-1C chain-like [KO:K07374]
105580388  tubulin alpha-1B chain [KO:K07374]
105574111  TUBA3E; LOW QUALITY PROTEIN: tubulin alpha-3E chain [KO:K07374]
105597442  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
105591250  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
105577167  tubulin beta-8 chain-like [KO:K07375]
105600022  TUBB; tubulin beta chain isoform X1 [KO:K07375]
105600115  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
105585313  TUBB1; tubulin beta-1 chain [KO:K07375]
105601184  tubulin gamma chain-like [KO:K07375]
105593855  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
105595229  TUBB3; tubulin beta-3 chain [KO:K07375]
105580625  tubulin beta chain [KO:K07375]
105580626  TUBB4B; tubulin beta-4B chain [KO:K07375]
105580651  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
105581289  tubulin beta-8 chain [KO:K07375]
105596432  TUBB2A; tubulin beta-2A chain [KO:K07375]
105596434  tubulin beta-2B chain isoform X1 [KO:K07375]
105598939  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
105591865  TUBB4A; tubulin beta-4A chain [KO:K07375]
105596855  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
105587256  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
105590052  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
105589891  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
105586090  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
105579138  DCTN6; LOW QUALITY PROTEIN: dynactin subunit 6 [KO:K10428]
105581884  ACTR1A; alpha-centractin [KO:K16575]
105583701  ACTR1B; beta-centractin [KO:K16575]
105586676  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
105579723  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
105576947  DNAH14; dynein heavy chain 14, axonemal isoform X1 [KO:K10408]
105584512  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
105592310  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
105577821  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
105585555  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
105585613  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
105585944  DNAH5; dynein heavy chain 5, axonemal isoform X1 [KO:K10408]
105586871  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
105579979  DNAH7; LOW QUALITY PROTEIN: dynein heavy chain 7, axonemal [KO:K10408]
105587632  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
105589517  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
105582192  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
105591018  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
105594745  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
105590058  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
105577835  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
105590830  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
105573064  DNAL4; dynein light chain 4, axonemal [KO:K10412]
105586801  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
105591502  NOS2; nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
105589917  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
105597886  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
105580611  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
105572154  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
105585127  GRIN2B; glutamate receptor ionotropic, NMDA 2B isoform X1 [KO:K05210]
105577760  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
105595236  GRIN2D; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D [KO:K05212]
105579123  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
105599874  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
105593696  pro-neuregulin-3, membrane-bound isoform-like [KO:K05457]
105593698  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
105601326  NRG4; pro-neuregulin-4, membrane-bound isoform isoform X1 [KO:K05458]
105580144  ERBB4; receptor tyrosine-protein kinase erbB-4 isoform X1 [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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J Cell Mol Med 15:2025-39 (2011)
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PMID:16472115
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Turner BJ, Atkin JD
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ER stress and UPR in familial amyotrophic lateral sclerosis.
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Curr Mol Med 6:79-86 (2006)
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ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
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Genes Dev 22:1451-64 (2008)
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PMID:21834058
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Walker AK, Atkin JD
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Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
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IUBMB Life 63:754-63 (2011)
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Renaud L, Picher-Martel V, Codron P, Julien JP
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Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
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Acta Neuropathol Commun 7:103 (2019)
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Trends Genet 34:404-423 (2018)
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Front Mol Neurosci 7:63 (2014)
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Sigma-1 receptor and amyotrophic lateral sclerosis.
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Neurology 91:743-747 (2018)
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Related
pathway
caty00190  Oxidative phosphorylation
caty03013  Nucleocytoplasmic transport
caty03050  Proteasome
caty04010  MAPK signaling pathway
caty04012  ErbB signaling pathway
caty04020  Calcium signaling pathway
caty04115  p53 signaling pathway
caty04137  Mitophagy - animal
caty04140  Autophagy - animal
caty04141  Protein processing in endoplasmic reticulum
caty04210  Apoptosis
caty04724  Glutamatergic synapse
caty04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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