KEGG   PATHWAY: cfr05014
Entry
cfr05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Camelus ferus (Wild Bactrian camel)
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
cfr05014  Amyotrophic lateral sclerosis
cfr05014

Organism
Camelus ferus (Wild Bactrian camel) [GN:cfr]
Gene
102524476  TP53; cellular tumor antigen p53 [KO:K04451]
102514999  CASP1; caspase-1 [KO:K01370] [EC:3.4.22.36]
102522363  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
102518434  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
102504996  BCL2L1; bcl-2-like protein 1 isoform X2 [KO:K04570]
102512073  ferritin light chain isoform X1 [KO:K02159]
102523295  BAD; bcl2-associated agonist of cell death isoform X2 [KO:K02158]
102516966  APAF1; apoptotic protease-activating factor 1 isoform X3 [KO:K02084]
102524306  cytochrome c [KO:K08738]
116663281  cytochrome c-like [KO:K08738]
116663681  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
102519816  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
102520133  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
102505386  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
102504600  LOW QUALITY PROTEIN: superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
5326219  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
5326218  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
5326226  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
5326228  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
5326227  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
5326229  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
5326221  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
102519984  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
102522489  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
102521191  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
102510141  LOW QUALITY PROTEIN: nucleolin 2-like [KO:K03944]
116662114  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
102523341  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
102515191  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
102515165  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
102516214  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
102523689  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
102510956  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
102512695  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
102505652  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
102510326  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
102509936  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
102512974  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
102504715  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
102518907  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
102520409  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12-like [KO:K11352]
102503878  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
102511004  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
116664480  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
116666587  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
102523839  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
102508877  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
102506336  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
102521082  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
102504305  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
102519500  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
102507693  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
102518585  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
102522078  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
102505428  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
102524292  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
102508476  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
102524104  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
102522662  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
102510949  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
102522433  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
102520779  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X2 [KO:K03941] [EC:7.1.1.2]
102522956  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
102517243  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
102523856  SDHA; LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
102524547  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
102506175  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
102521513  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
102517471  cytochrome b-c1 complex subunit Rieske, mitochondrial isoform X1 [KO:K00411] [EC:7.1.1.8]
5326230  CYTB; cytochrome b [KO:K00412]
102523866  cytochrome c1, heme protein, mitochondrial [KO:K00413]
102514495  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
102515646  cytochrome b-c1 complex subunit 2, mitochondrial isoform X1 [KO:K00415]
102504726  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
102511190  cytochrome b-c1 complex subunit 7 [KO:K00417]
102520672  cytochrome b-c1 complex subunit 8 [KO:K00418]
102514673  cytochrome b-c1 complex subunit 8-like [KO:K00418]
102522632  cytochrome b-c1 complex subunit 8 [KO:K00418]
102523993  cytochrome b-c1 complex subunit 9 [KO:K00419]
102505913  cytochrome b-c1 complex subunit 10 [KO:K00420]
5326220  COX3; cytochrome c oxidase subunit III [KO:K02262]
5326222  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
5326224  COX2; cytochrome c oxidase subunit II [KO:K02261]
102504739  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
102506135  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
102514933  cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
102506544  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
102524231  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
102515110  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
102517088  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
102512681  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
102511501  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
102506051  cytochrome c oxidase subunit 6B1 [KO:K02267]
116666021  cytochrome c oxidase subunit 6B2 [KO:K02267]
102514870  cytochrome c oxidase subunit 6C [KO:K02268]
102507060  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
102509827  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
102511089  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
102509421  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
102506447  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
116662174  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
116666513  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
102514229  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
102513074  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
102505103  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
102517399  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
116657863  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
5326225  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
102515424  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
102512235  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
102512003  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
102508921  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
102518323  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
102515952  ATP5PO; LOW QUALITY PROTEIN: ATP synthase subunit O, mitochondrial [KO:K02137]
102507559  LOW QUALITY PROTEIN: ATP synthase subunit O, mitochondrial-like [KO:K02137]
102506489  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
5326223  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
102518825  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
116669325  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
102507566  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
102504032  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
102507581  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
102521586  PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
102508809  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
102505918  OPTN; optineurin isoform X1 [KO:K19946]
102519914  SQSTM1; sequestosome-1 [KO:K14381]
102509665  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
102505462  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
102514948  TBK1; serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
102517502  TANK; TRAF family member-associated NF-kappa-B activator isoform X3 [KO:K12650]
102521985  WDR41; WD repeat-containing protein 41 [KO:K23610]
102506594  SMCR8; LOW QUALITY PROTEIN: guanine nucleotide exchange protein SMCR8 [KO:K23611]
102509537  C9orf72; guanine nucleotide exchange C9orf72 homolog [KO:K23609]
102517974  RAB8A; ras-related protein Rab-8A [KO:K07901]
102513155  RAB39B; ras-related protein Rab-39B [KO:K07925]
102509266  RAB1A; ras-related protein Rab-1A [KO:K07874]
102510836  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
102508563  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
102516695  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
102509718  ATG101; autophagy-related protein 101 isoform X2 [KO:K19730]
102513895  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
102518316  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
102512599  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
102515881  BECN1; beclin-1 isoform X3 [KO:K08334]
102517297  BECN2; beclin-2 [KO:K08334]
102508821  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
102522384  ATG14; beclin 1-associated autophagy-related key regulator isoform X1 [KO:K17889]
102515126  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
102503733  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
102524532  ATG2B; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B [KO:K17906]
102521302  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
102515887  WIPI2; LOW QUALITY PROTEIN: WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
102503459  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
102506577  ALS2; alsin isoform X1 [KO:K04575]
102515139  RAB5A; ras-related protein Rab-5A [KO:K07887]
102516893  RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392]
102510627  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
102510940  FIG4; polyphosphoinositide phosphatase [KO:K22913] [EC:3.1.3.-]
102503712  spatacsin isoform X1 [KO:K19026]
102513924  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
102515660  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
102505131  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
106729683  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
116669020  PPP3R1; calcineurin subunit B type 1 [KO:K06268]
102520314  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
102510376  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
102505540  UBQLN3; ubiquilin-3 [KO:K04523]
102519942  UBQLN2; ubiquilin-2 [KO:K04523]
102508944  VCP; transitional endoplasmic reticulum ATPase [KO:K13525]
102522634  DERL1; derlin-1 isoform X1 [KO:K11519]
102516292  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
102505753  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
102517648  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
102504963  PSMA7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
102506822  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
102523817  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
102521944  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
102513698  LOW QUALITY PROTEIN: proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
102509508  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
102521312  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
102518943  PSMB7; proteasome subunit beta type-7 isoform X1 [KO:K02739] [EC:3.4.25.1]
102504556  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
102512214  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
102517423  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
102523305  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
102519021  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
102513574  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
102519658  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
102504898  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
102508776  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
102506679  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
102517432  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
116656643  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
102520605  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
102516065  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
102523988  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X2 [KO:K06693]
102521709  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
102513989  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
102510119  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
102513652  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
102509607  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X2 [KO:K03039]
102516967  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
102517910  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
102515414  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
102517207  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 8-like [KO:K03031]
102504204  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
102517349  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
102507599  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
102510362  TARDBP; TAR DNA-binding protein 43 [KO:K23600]
102513734  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
102513343  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
102508147  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
102504126  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X2 [KO:K13158]
102510499  heterogeneous nuclear ribonucleoproteins A2/B1 [KO:K13158]
102522398  LOW QUALITY PROTEIN: polyadenylate-binding protein, cytoplasmic and nuclear [KO:K13158]
102510277  ATXN2; ataxin-2 isoform X1 [KO:K23625]
102511383  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
102520593  MATR3; matrin-3 isoform X1 [KO:K13213]
102507917  ANG; angiogenin [KO:K16631] [EC:3.1.27.-]
102512866  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
102507187  ALYREF; THO complex subunit 4 [KO:K12881]
102509658  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
102508268  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
102516338  LOW QUALITY PROTEIN: nuclear RNA export factor 3 [KO:K14284]
102522387  NXF1; nuclear RNA export factor 1 [KO:K14284]
102514126  nuclear RNA export factor 2 [KO:K14284]
116662257  nuclear RNA export factor 3-like isoform X1 [KO:K14284]
102513088  NXT1; NTF2-related export protein 1 [KO:K14285]
102512919  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
102512769  GLE1; nucleoporin GLE1 isoform X1 [KO:K18723]
102510769  TPR; LOW QUALITY PROTEIN: nucleoprotein TPR [KO:K09291]
102515214  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
102514624  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
102512605  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
102512531  RAE1; mRNA export factor [KO:K14298]
102512636  SEC13; protein SEC13 homolog [KO:K14004]
102503848  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
102507172  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
102523774  NUP107; nuclear pore complex protein Nup107 isoform X2 [KO:K14301]
102507882  NUP37; nucleoporin Nup37 [KO:K14302]
102508723  NUP160; nuclear pore complex protein Nup160 [KO:K14303]
102517427  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
102517390  NUP43; nucleoporin Nup43 [KO:K14305]
102503784  NUP62; LOW QUALITY PROTEIN: nuclear pore glycoprotein p62 [KO:K14306]
102517887  NUP62CL; nucleoporin-62 C-terminal-like protein [KO:K14306]
102506601  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
102521801  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
102506943  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
102523676  NUP205; nuclear pore complex protein Nup205 [KO:K14310]
102506384  NUP188; nucleoporin NUP188 homolog isoform X1 [KO:K14311]
102504412  NUP155; nuclear pore complex protein Nup155 [KO:K14312]
102515360  NUP35; nucleoporin NUP35 [KO:K14313]
102517834  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
102507321  NUP210; LOW QUALITY PROTEIN: nuclear pore membrane glycoprotein 210 [KO:K14314]
102519228  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
102505938  LOW QUALITY PROTEIN: nuclear envelope pore membrane protein POM 121C-like [KO:K14316]
102511555  nuclear envelope pore membrane protein POM 121C [KO:K14316]
102519934  POM121L2; POM121-like protein 2 [KO:K14316]
102520268  NUP214; nuclear pore complex protein Nup214 isoform X2 [KO:K14317]
102505164  NUP88; nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
102514604  E3 SUMO-protein ligase RanBP2 isoform X1 [KO:K12172] [EC:2.3.2.-]
102522476  SETX; probable helicase senataxin [KO:K10706] [EC:5.6.2.5 5.6.2.3]
106728716  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
102515954  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
102511320  VAPB; vesicle-associated membrane protein-associated protein B/C [KO:K10707]
102515280  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
102517621  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
102518977  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
102510269  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
102511649  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
102522243  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
102523410  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
102518617  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
102508076  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
102524078  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960]
102523406  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
102510424  TNF; tumor necrosis factor [KO:K03156]
102508884  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A isoform X1 [KO:K03158]
102507931  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
102503654  DAXX; death domain-associated protein 6 [KO:K02308]
102523780  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
102519784  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 [KO:K04433] [EC:2.7.12.2]
102516847  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
102519781  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
102511298  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
102511078  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
102508946  NOS1; nitric oxide synthase, brain isoform X1 [KO:K13240] [EC:1.14.13.39]
102504999  CCS; copper chaperone for superoxide dismutase [KO:K04569]
102523217  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
102509450  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
102508398  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
102515413  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
102505123  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
102517670  GPX6; glutathione peroxidase 6 isoform X1 [KO:K00432] [EC:1.11.1.9]
102517908  epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
102524165  CAT; catalase [KO:K03781] [EC:1.11.1.6]
102523724  PRPH; peripherin isoform X1 [KO:K07607]
102513324  NEFL; neurofilament light polypeptide [KO:K04572]
102513071  NEFM; neurofilament medium polypeptide isoform X1 [KO:K04573]
102505093  NEFH; neurofilament heavy polypeptide isoform X1 [KO:K04574]
102512763  PFN1; LOW QUALITY PROTEIN: profilin-1 [KO:K05759]
102510311  PFN3; profilin-3 [KO:K05759]
102524451  uncharacterized protein LOC102524451 [KO:K05759]
102515046  PFN4; profilin-4 [KO:K05759]
102508943  PFN2; profilin-2 isoform X1 [KO:K05759]
102514032  ACTB; actin, cytoplasmic 1 [KO:K05692]
102509393  actin, cytoplasmic 1 [KO:K05692]
102516333  LOW QUALITY PROTEIN: actin, clone 302 [KO:K05692]
116660822  actin, cytoplasmic 1-like isoform X1 [KO:K05692]
102519581  ACTG1; actin, cytoplasmic 2 [KO:K05692]
102513281  ANXA7; annexin A7 isoform X3 [KO:K17095]
102508258  ANXA11; annexin A11 [KO:K17095]
102518661  KIF5A; kinesin heavy chain isoform X3 [KO:K10396]
102517013  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
102519328  KIF5C; kinesin heavy chain [KO:K10396]
102510359  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
102511669  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
102523618  KLC3; kinesin light chain 3 isoform X3 [KO:K10407]
102509206  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
102522963  tubulin alpha-1B chain [KO:K07374]
102523472  TUBA1A; tubulin alpha-1A chain [KO:K07374]
102515991  tubulin alpha-1C chain [KO:K07374]
102521054  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
102522237  TUBA4A; tubulin alpha-4A chain [KO:K07374]
102512533  LOW QUALITY PROTEIN: tubulin alpha-1D chain [KO:K07374]
102508381  tubulin alpha chain [KO:K07374]
102505604  LOW QUALITY PROTEIN: tubulin alpha-1A chain [KO:K07374]
102518229  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
102505031  tubulin alpha-3 chain [KO:K07374]
102511248  TUBB3; tubulin beta-3 chain [KO:K07375]
102514505  TUBB2A; LOW QUALITY PROTEIN: tubulin beta-2A chain [KO:K07375]
102514940  LOW QUALITY PROTEIN: tubulin beta-2B chain [KO:K07375]
102514277  TUBB1; tubulin beta-1 chain [KO:K07375]
102522960  TUBB4A; tubulin beta-4A chain [KO:K07375]
102511603  TUBB4B; LOW QUALITY PROTEIN: tubulin beta-4B chain [KO:K07375]
102523678  TUBB6; tubulin beta-6 chain [KO:K07375]
102506772  TUBB; tubulin beta chain isoform X2 [KO:K07375]
102505844  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
102518272  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
102522650  DCTN3; dynactin subunit 3 [KO:K10425]
102510178  DCTN4; dynactin subunit 4 [KO:K10426]
102512500  DCTN5; dynactin subunit 5 [KO:K10427]
102519298  DCTN6; dynactin subunit 6 [KO:K10428]
102523981  ACTR1B; beta-centractin isoform X1 [KO:K16575]
102517533  ACTR1A; alpha-centractin [KO:K16575]
102509264  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
102518988  HAP1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
102518700  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
102509803  DNAH5; LOW QUALITY PROTEIN: dynein heavy chain 5, axonemal [KO:K10408]
102507171  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
102513082  DNAH9; LOW QUALITY PROTEIN: dynein heavy chain 9, axonemal [KO:K10408]
102516457  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
102518470  DNAH8; LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal [KO:K10408]
102520086  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
102513136  DNAH17; LOW QUALITY PROTEIN: dynein heavy chain 17, axonemal [KO:K10408]
102511164  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
102508411  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
102506868  DNAH3; LOW QUALITY PROTEIN: dynein heavy chain 3, axonemal [KO:K10408]
102514536  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
102518110  DNAH11; LOW QUALITY PROTEIN: dynein heavy chain 11, axonemal [KO:K10408]
102518938  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
102521975  DNAI1; LOW QUALITY PROTEIN: dynein intermediate chain 1, axonemal [KO:K10409]
102514425  DNAI2; dynein intermediate chain 2, axonemal isoform X1 [KO:K11143]
102504852  DNAL1; dynein light chain 1, axonemal [KO:K10411]
102509708  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
102522035  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
102518523  NOS2; nitric oxide synthase, inducible isoform X1 [KO:K13241] [EC:1.14.13.39]
102513727  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
102509459  GRIA2; LOW QUALITY PROTEIN: glutamate receptor 2 [KO:K05198]
102506181  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
102503788  GRIN2A; glutamate receptor ionotropic, NMDA 2A [KO:K05209]
102517500  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
102516268  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
102522985  GRIN2D; glutamate receptor ionotropic, NMDA 2D isoform X1 [KO:K05212]
102510288  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X5 [KO:K05455]
102510636  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
102514418  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
102522168  NRG4; pro-neuregulin-4, membrane-bound isoform isoform X1 [KO:K05458]
102522654  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
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
cfr00190  Oxidative phosphorylation
cfr03013  Nucleocytoplasmic transport
cfr03050  Proteasome
cfr04010  MAPK signaling pathway
cfr04012  ErbB signaling pathway
cfr04020  Calcium signaling pathway
cfr04115  p53 signaling pathway
cfr04137  Mitophagy - animal
cfr04140  Autophagy - animal
cfr04141  Protein processing in endoplasmic reticulum
cfr04210  Apoptosis
cfr04724  Glutamatergic synapse
cfr04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

DBGET integrated database retrieval system