KEGG   PATHWAY: ray05014
Entry
ray05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Rousettus aegyptiacus (Egyptian rousette)
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
ray05014  Amyotrophic lateral sclerosis
ray05014

Organism
Rousettus aegyptiacus (Egyptian rousette) [GN:ray]
Gene
107515689  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
107504293  CASP1; caspase-1 isoform X1 [KO:K01370] [EC:3.4.22.36]
107500447  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
107503915  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
107512887  BCL2L1; bcl-2-like protein 1 [KO:K04570]
107498831  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
107497091  BAD; bcl2-associated agonist of cell death [KO:K02158]
107517482  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
107518128  cytochrome c [KO:K08738]
107501830  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
107510456  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
107519576  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
3666157  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
3666158  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
3666159  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
3666160  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
3666161  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
3666162  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
3666163  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
107503551  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
107498174  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
107510187  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944]
107521562  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
107503054  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
107518757  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
107501488  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
107500215  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
107505569  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
107519918  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951]
107516331  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
107513442  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
107510586  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
107516106  NDUFAB1; LOW QUALITY PROTEIN: acyl carrier protein, mitochondrial [KO:K03955]
107510995  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
107519774  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
107503701  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
107511238  NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
107504059  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
107503161  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
107517762  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
107501648  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
107502802  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
107519420  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
107499769  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
107521586  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
107506067  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
107501551  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
107505180  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
107504677  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
107511837  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
107509658  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
107517069  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
107499974  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
107500941  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
107503528  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
107515342  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
107519203  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
107499960  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
107501827  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
107504683  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
107512967  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
107515880  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
3666156  CYTB; cytochrome b [KO:K00412]
107503471  cytochrome c1, heme protein, mitochondrial [KO:K00413]
107513320  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
107516078  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
107517907  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
107500346  uncharacterized protein LOC107500346 isoform X1 [KO:K00417]
107497034  cytochrome b-c1 complex subunit 8 [KO:K00418]
107520492  cytochrome b-c1 complex subunit 9 [KO:K00419]
107500917  cytochrome b-c1 complex subunit 10 [KO:K00420]
3666155  COX3; cytochrome c oxidase subunit III [KO:K02262]
3666153  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
3666154  COX2; cytochrome c oxidase subunit II [KO:K02261]
107502351  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
107512889  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
107510524  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
107509970  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
107505626  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
107501251  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
107513145  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6B1-like [KO:K02267]
107515431  cytochrome c oxidase subunit 6B2 [KO:K02267]
107497665  cytochrome c oxidase subunit 6B1 [KO:K02267]
107511938  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6C-like [KO:K02268]
107507599  cytochrome c oxidase subunit 6C [KO:K02268]
107509286  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
107509593  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
107497645  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
107503849  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
107519004  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
107498048  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
107497080  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
107512344  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
107521221  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
107505971  ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
107500926  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
107500091  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
3666151  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
107516139  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
107509866  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
107504969  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
107503791  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
107520368  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
107508540  ATP5J; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
3666152  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
107506795  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
107513476  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
107504679  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
107516793  calcium uniporter protein, mitochondrial [KO:K20858]
107513041  PINK1; serine/threonine-protein kinase PINK1, mitochondrial [KO:K05688] [EC:2.7.11.1]
107501685  PARK2; LOW QUALITY PROTEIN: E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
107504847  OPTN; optineurin isoform X1 [KO:K19946]
107512321  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
107502341  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
107507227  LOW QUALITY PROTEIN: microtubule-associated proteins 1A/1B light chain 3C-like [KO:K10435]
107500801  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
107508222  TBK1; serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
107499880  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
107501525  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
107511080  SMCR8; Smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611]
107516484  protein C9orf72 homolog [KO:K23609]
107517240  RAB8A; ras-related protein Rab-8A [KO:K07901]
107504341  RAB39B; ras-related protein Rab-39B [KO:K07925]
107518312  RAB1A; ras-related protein Rab-1A [KO:K07874]
107513109  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
107511543  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
107511063  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
107509824  ATG101; autophagy-related protein 101 isoform X1 [KO:K19730]
107511859  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
107502095  RB1CC1; RB1-inducible coiled-coil protein 1 [KO:K17589]
107511862  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
107513845  BECN1; beclin-1 [KO:K08334]
107497279  BECN2; beclin-2 [KO:K08334]
107508463  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
107510917  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
107517970  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
107500133  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
107517101  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
107518323  ATG2B; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B [KO:K17906]
107510293  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
107505308  WIPI2; LOW QUALITY PROTEIN: WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
107503166  ALS2; alsin isoform X1 [KO:K04575]
107497819  RAB5A; ras-related protein Rab-5A [KO:K07887]
107515925  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
107511461  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
107505358  polyphosphoinositide phosphatase-like [KO:K22913] [EC:3.1.3.-]
107519380  FIG4; polyphosphoinositide phosphatase [KO:K22913] [EC:3.1.3.-]
107505834  SPG11; spatacsin isoform X1 [KO:K19026]
107516843  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
107517885  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
107509227  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
107506933  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
107499586  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
107521518  UBQLN4; ubiquilin-4 [KO:K04523]
107502806  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
107521927  UBQLN2; ubiquilin-2 [KO:K04523]
107501057  ubiquilin-3-like [KO:K04523]
107502827  VCP; LOW QUALITY PROTEIN: transitional endoplasmic reticulum ATPase [KO:K13525]
107503228  DERL1; LOW QUALITY PROTEIN: derlin-1 [KO:K11519]
107514414  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
107497282  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
107500521  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
107503668  PSMA7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
107518428  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
107516149  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
107519400  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
107502125  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
107509459  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
107506651  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
107520678  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
107512091  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
107498041  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
107500159  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
107499048  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
107511508  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
107515474  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
107521811  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
107498130  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
107511840  PSMC3; 26S protease regulatory subunit 6A [KO:K03065]
107501981  PSMC5; 26S protease regulatory subunit 8 isoform X1 [KO:K03066]
107504916  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
107504976  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
107520710  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
107515236  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
107502179  PSMD12; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
107514642  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
107513474  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
107511919  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
107506504  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
107498998  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
107508548  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
107499882  26S proteasome non-ATPase regulatory subunit 14-like [KO:K03030]
107520970  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
107503669  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
107504477  SHFM1; 26S proteasome complex subunit DSS1 [KO:K10881]
107515022  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
107513107  TARDBP; TAR DNA-binding protein 43 [KO:K23600]
107503992  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
107521075  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
107508350  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
107514670  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
107514871  heterogeneous nuclear ribonucleoprotein A1-like 2 [KO:K12741]
107500742  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
107505661  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
107513252  ATXN2; ataxin-2 isoform X1 [KO:K23625]
107515800  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
107506226  MATR3; matrin-3 isoform X1 [KO:K13213]
107521161  ANG; angiogenin isoform X1 [KO:K16631] [EC:3.1.27.-]
107498892  angiogenin-like [KO:K16631] [EC:3.1.27.-]
107511680  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
107520286  ALYREF; THO complex subunit 4 [KO:K12881]
107514680  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
107519534  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
107519684  NXF3; nuclear RNA export factor 3 isoform X1 [KO:K14284]
107506262  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
107501584  NXF1; nuclear RNA export factor 1 [KO:K14284]
107519301  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
107498637  NXT1; NTF2-related export protein 1 [KO:K14285]
107500796  GLE1; nucleoporin GLE1 isoform X1 [KO:K18723]
107521613  TPR; nucleoprotein TPR isoform X1 [KO:K09291]
107505526  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
107516274  NUP153; LOW QUALITY PROTEIN: nuclear pore complex protein Nup153 [KO:K14296]
107517364  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
107500089  RAE1; LOW QUALITY PROTEIN: mRNA export factor [KO:K14298]
107498352  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
107497711  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
107520058  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
107512677  NUP107; nuclear pore complex protein Nup107 [KO:K14301]
107501178  NUP37; nucleoporin Nup37 [KO:K14302]
107511827  NUP160; nuclear pore complex protein Nup160 [KO:K14303]
107503775  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
107518461  NUP43; nucleoporin Nup43 [KO:K14305]
107498601  NUP62CL; nucleoporin-62 C-terminal-like protein isoform X1 [KO:K14306]
107500429  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
107520894  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
107516363  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
107499657  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
107498236  NUP205; nuclear pore complex protein Nup205 [KO:K14310]
107504522  NUP188; nucleoporin NUP188 homolog isoform X1 [KO:K14311]
107509718  NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
107513281  NUP35; nucleoporin NUP53 isoform X1 [KO:K14313]
107505419  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
107509173  NUP210L; nuclear pore membrane glycoprotein 210-like isoform X1 [KO:K14314]
107497948  NDC1; nucleoporin NDC1 [KO:K14315]
107504644  POM121L2; POM121-like protein 2 [KO:K14316]
107511807  POM121C; nuclear envelope pore membrane protein POM 121C isoform X1 [KO:K14316]
107504554  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
107504493  nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
107509448  nuclear pore complex protein Nup88-like [KO:K14318]
107519863  RANBP2; E3 SUMO-protein ligase RanBP2 isoform X1 [KO:K12172] [EC:2.3.2.-]
107504565  SETX; LOW QUALITY PROTEIN: probable helicase senataxin [KO:K10706] [EC:5.6.2.5 5.6.2.3]
107501267  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
107506635  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
107500046  VAPB; vesicle-associated membrane protein-associated protein B/C [KO:K10707]
107502472  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
107501502  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
107502023  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107520486  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
107503081  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
107521291  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
107504291  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
107512376  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
107503258  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
107500816  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
107511965  ITPR3; LOW QUALITY PROTEIN: inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
107502870  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
107498497  TNF; tumor necrosis factor [KO:K03156]
107519788  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A isoform X1 [KO:K03158]
107513133  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B [KO:K05141]
107511976  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
107506757  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
107510279  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
107497883  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
107497895  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
107500264  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
107500274  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
107513162  NOS1; LOW QUALITY PROTEIN: nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
107513863  CCS; copper chaperone for superoxide dismutase [KO:K04569]
107509312  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
107509647  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
107504238  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
107513349  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
107505650  LOW QUALITY PROTEIN: glutathione peroxidase 1-like [KO:K00432] [EC:1.11.1.9]
107497149  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
107497960  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
107504104  CAT; catalase [KO:K03781] [EC:1.11.1.6]
107514244  PRPH; peripherin [KO:K07607]
107512531  NEFL; neurofilament light polypeptide [KO:K04572]
107512532  NEFM; neurofilament medium polypeptide [KO:K04573]
107520482  NEFH; LOW QUALITY PROTEIN: neurofilament heavy polypeptide [KO:K04574]
107517535  PFN3; profilin-3 [KO:K05759]
107516596  PFN2; profilin-2 isoform X1 [KO:K05759]
107509489  PFN1; profilin-1 [KO:K05759]
107499322  profilin-1-like [KO:K05759]
107498552  PFN4; profilin-4 isoform X1 [KO:K05759]
107520262  ACTG1; actin, cytoplasmic 2 [KO:K05692]
107515934  ACTB; actin, cytoplasmic 1 [KO:K05692]
107520804  ANXA11; annexin A11 isoform X1 [KO:K17095]
107516848  ANXA7; annexin A7 isoform X1 [KO:K17095]
107513931  KIF5C; kinesin heavy chain isoform 5C [KO:K10396]
107518439  KIF5B; kinesin-1 heavy chain [KO:K10396]
107501516  KIF5A; kinesin heavy chain isoform 5A isoform X1 [KO:K10396]
107513627  KLC3; kinesin light chain 3 [KO:K10407]
107497533  KLC2; kinesin light chain 2 [KO:K10407]
107498378  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
107501964  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
107516782  tubulin alpha-1B chain-like [KO:K07374]
107500328  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
107497847  LOW QUALITY PROTEIN: tubulin alpha-3 chain-like [KO:K07374]
107497857  tubulin alpha-3 chain-like [KO:K07374]
107514242  tubulin alpha-1A chain isoform X1 [KO:K07374]
107514243  tubulin alpha-1C chain [KO:K07374]
107516732  tubulin alpha-4A chain isoform X1 [KO:K07374]
107502098  tubulin beta-3 chain [KO:K07375]
107503105  TUBB4B; LOW QUALITY PROTEIN: tubulin beta-4B chain [KO:K07375]
107511148  TUBB4A; tubulin beta-4A chain [KO:K07375]
107521721  TUBB2A; tubulin beta-2A chain [KO:K07375]
107521722  tubulin beta-2B chain [KO:K07375]
107500090  TUBB1; LOW QUALITY PROTEIN: tubulin beta-1 chain [KO:K07375]
107497724  TUBB6; tubulin beta-6 chain [KO:K07375]
107498082  TUBB; tubulin beta chain [KO:K07375]
107502898  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
107501501  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
107502859  DCTN3; dynactin subunit 3 [KO:K10425]
107497166  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
107516090  DCTN5; dynactin subunit 5 [KO:K10427]
107505788  DCTN6; dynactin subunit 6 [KO:K10428]
107515575  ACTR1A; alpha-centractin [KO:K16575]
107509969  ACTR1B; beta-centractin isoform X1 [KO:K16575]
107502129  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
107503393  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
107504601  DNAH10; LOW QUALITY PROTEIN: dynein heavy chain 10, axonemal [KO:K10408]
107498566  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
107506355  DNAH6; LOW QUALITY PROTEIN: dynein heavy chain 6, axonemal [KO:K10408]
107514440  DNAH5; LOW QUALITY PROTEIN: dynein heavy chain 5, axonemal [KO:K10408]
107515685  DNAH2; dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
107515695  LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal-like [KO:K10408]
107508404  DNAH7; LOW QUALITY PROTEIN: dynein heavy chain 7, axonemal [KO:K10408]
107510065  DNAH17; LOW QUALITY PROTEIN: dynein heavy chain 17, axonemal [KO:K10408]
107518131  DNAH11; dynein heavy chain 11, axonemal isoform X1 [KO:K10408]
107515470  dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
107517402  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
107516076  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
107521068  DNAH14; LOW QUALITY PROTEIN: dynein heavy chain 14, axonemal [KO:K10408]
107517072  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
107502815  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
107510260  DNAI2; LOW QUALITY PROTEIN: dynein intermediate chain 2, axonemal [KO:K11143]
107505020  DNAL1; dynein light chain 1, axonemal [KO:K10411]
107519300  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
107499243  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
107497410  NOS2; nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
107521882  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
107507556  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
107503090  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
107514651  GRIN2A; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2A [KO:K05209]
107508606  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
107503801  GRIN2C; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2C [KO:K05211]
107498864  GRIN2D; LOW QUALITY PROTEIN: glutamate receptor ionotropic, NMDA 2D [KO:K05212]
107505812  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
107521551  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
107498386  NRG3; LOW QUALITY PROTEIN: pro-neuregulin-3, membrane-bound isoform [KO:K05457]
107517636  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
Reference
  Authors
Brown RH, Al-Chalabi A
  Title
Amyotrophic Lateral Sclerosis.
  Journal
N Engl J Med 377:162-172 (2017)
DOI:10.1056/NEJMra1603471
Reference
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  Authors
Hand CK, Rouleau GA
  Title
Familial amyotrophic lateral sclerosis.
  Journal
Muscle Nerve 25:135-59 (2002)
DOI:10.1002/mus.10000
Reference
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  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
  Authors
Benarroch EE
  Title
Sigma-1 receptor and amyotrophic lateral sclerosis.
  Journal
Neurology 91:743-747 (2018)
DOI:10.1212/WNL.0000000000006347
Reference
  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
ray00190  Oxidative phosphorylation
ray03013  Nucleocytoplasmic transport
ray03050  Proteasome
ray04010  MAPK signaling pathway
ray04012  ErbB signaling pathway
ray04020  Calcium signaling pathway
ray04115  p53 signaling pathway
ray04137  Mitophagy - animal
ray04140  Autophagy - animal
ray04141  Protein processing in endoplasmic reticulum
ray04210  Apoptosis
ray04724  Glutamatergic synapse
ray04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

DBGET integrated database retrieval system