KEGG   PATHWAY: cimi05014
Entry
cimi05014                   Pathway                                
Name
Amyotrophic lateral sclerosis - Cebus imitator (Panamanian white-faced capuchin)
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
cimi05014  Amyotrophic lateral sclerosis
cimi05014

Organism
Cebus imitator (Panamanian white-faced capuchin) [GN:cimi]
Gene
108306559  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
108315450  CASP1; caspase-1 isoform X2 [KO:K01370] [EC:3.4.22.36]
108294856  BID; BH3-interacting domain death agonist [KO:K04726]
108304605  BCL2; apoptosis regulator Bcl-2 isoform X2 [KO:K02161]
108293471  BCL2L1; bcl-2-like protein 1 [KO:K04570]
108287107  apoptosis regulator BAX-like [KO:K02159]
108283911  BAX; apoptosis regulator BAX [KO:K02159]
108310302  BAD; bcl2-associated agonist of cell death isoform X1 [KO:K02158]
108290860  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
108281951  cytochrome c, somatic-like [KO:K08738]
108288191  cytochrome c-like [KO:K08738]
108288636  cytochrome c-like [KO:K08738]
108299683  cytochrome c-like [KO:K08738]
108290168  cytochrome c [KO:K08738]
108290453  cytochrome c-like [KO:K08738]
108303234  cytochrome c [KO:K08738]
108305303  cytochrome c, somatic-like [KO:K08738]
108306377  cytochrome c, somatic-like [KO:K08738]
108311641  cytochrome c, testis-specific-like [KO:K08738]
108304381  cytochrome c-like [KO:K08738]
108313879  cytochrome c-like [KO:K08738]
108293847  cytochrome c [KO:K08738]
108305848  cytochrome c-like [KO:K08738]
108307291  cytochrome c-like [KO:K08738]
108286842  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
108288114  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
108310786  caspase-3-like [KO:K02187] [EC:3.4.22.56]
108295126  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
108310187  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
108314835  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
108311216  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
108298418  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
108291046  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
108303153  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
108310744  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
108296115  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
108310101  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
108315252  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
108282169  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
108289277  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
108293421  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
119468946  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
108305952  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
108281827  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108282800  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108309719  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
108288820  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108289584  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108301881  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
119471120  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108316955  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
108317568  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
108288663  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
119473819  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
108290471  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
108304352  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6-like [KO:K03950]
108283641  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
108285862  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
108309991  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
108296052  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
108308526  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
108289302  acyl carrier protein, mitochondrial-like [KO:K03955]
108302396  acyl carrier protein, mitochondrial-like [KO:K03955]
108304153  acyl carrier protein, mitochondrial-like [KO:K03955]
108311510  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
108304245  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
108294133  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
108284596  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
119467181  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
108301529  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
108283416  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
108287591  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
108291574  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
108302357  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
108315747  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
108292544  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
108314371  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
108287738  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
108317671  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
108316416  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6-like [KO:K03962]
108297603  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
108306362  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
108305986  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
108290944  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
108282471  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial-like [KO:K11351]
108295527  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
108310134  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
108293610  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
108304836  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
108307761  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
108288862  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
108290757  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
108291363  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
108294188  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
108293147  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
108310175  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
108305631  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial-like [KO:K03941] [EC:7.1.1.2]
108285589  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
108296268  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
108297730  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
108287259  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
108282309  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
108313212  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
108293638  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
108295470  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
108283532  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
108291317  cytochrome c1, heme protein, mitochondrial [KO:K00413]
108291340  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
108308500  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
108304212  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
108294769  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
108312782  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
108288619  cytochrome b-c1 complex subunit 7 [KO:K00417]
108289059  cytochrome b-c1 complex subunit 8-like [KO:K00418]
108307160  cytochrome b-c1 complex subunit 8 [KO:K00418]
108283344  cytochrome b-c1 complex subunit 9 [KO:K00419]
108315695  cytochrome b-c1 complex subunit 9-like [KO:K00419]
108304531  cytochrome b-c1 complex subunit 10-like [KO:K00420]
108293152  cytochrome b-c1 complex subunit 10 [KO:K00420]
108293503  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
108314664  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
108296976  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
108300479  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
108301038  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
108318442  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
108292179  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
108303559  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
108312070  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
108293926  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
108309225  cytochrome c oxidase subunit 6B1-like [KO:K02267]
108286354  uncharacterized protein LOC108286354 [KO:K02267]
108316623  cytochrome c oxidase subunit 6B2 [KO:K02267]
108281471  cytochrome c oxidase subunit 6C-like [KO:K02268]
108312576  cytochrome c oxidase subunit 6C-like [KO:K02268]
108286901  cytochrome c oxidase subunit 6C [KO:K02268]
108295926  cytochrome c oxidase subunit 6C-like [KO:K02268]
108287918  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
108301236  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
119471644  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
108308963  cytochrome c oxidase subunit 7A-related protein, mitochondrial-like [KO:K02270]
108315514  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
108297237  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
108292295  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
108312415  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
108288406  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108299095  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108284587  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108288888  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108310382  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
119472552  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108290000  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108298758  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
108294894  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
108316858  uncharacterized protein LOC108316858 isoform X1 [KO:K02273]
108305148  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
108309697  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
108298462  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
108309123  LOW QUALITY PROTEIN: ATP synthase subunit gamma, mitochondrial-like [KO:K02136]
108293174  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
108285927  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
108283157  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
108298729  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
119467521  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
108311019  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
108312260  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
108301979  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
119470894  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
108306059  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
108307096  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
108284890  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
108309400  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
108315392  ATP synthase subunit d, mitochondrial-like [KO:K02138]
108299416  ATP synthase subunit O, mitochondrial-like [KO:K02137]
108302528  ATP5PO; ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
108287657  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
108289843  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
108313501  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
108307148  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
108293613  TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518]
108285323  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
108300590  LOW QUALITY PROTEIN: calcium uniporter protein, mitochondrial-like [KO:K20858]
108296146  MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858]
108287839  serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
108310303  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
108305266  OPTN; optineurin isoform X1 [KO:K19946]
108295214  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
108292805  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
108314631  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
108306951  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
108288399  TBK1; serine/threonine-protein kinase TBK1 [KO:K05410] [EC:2.7.11.10]
108317391  TANK; TRAF family member-associated NF-kappa-B activator isoform X2 [KO:K12650]
108303747  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
108293472  SMCR8; guanine nucleotide exchange protein SMCR8 [KO:K23611]
108289450  C9orf72; guanine nucleotide exchange C9orf72 homolog [KO:K23609]
108284461  RAB8A; ras-related protein Rab-8A [KO:K07901]
108294370  RAB39B; ras-related protein Rab-39B [KO:K07925]
108299701  RAB1A; ras-related protein Rab-1A [KO:K07874]
108285700  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
108292658  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
108293490  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
108299074  autophagy-related protein 101-like [KO:K19730]
108316111  ATG101; autophagy-related protein 101 [KO:K19730]
108304864  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
108306695  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
108304868  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
108289803  BECN1; beclin-1 isoform X1 [KO:K08334]
108306883  BECN2; beclin-2 [KO:K08334]
108290361  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
108294063  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
108301025  nuclear receptor-binding factor 2-like [KO:K21246]
108285562  LOW QUALITY PROTEIN: nuclear receptor-binding factor 2-like [KO:K21246]
108305170  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
108310270  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
108304112  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
108308095  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
108281663  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
108295970  ALS2; alsin isoform X1 [KO:K04575]
108299936  RAB5A; ras-related protein Rab-5A [KO:K07887]
108303482  ras-related C3 botulinum toxin substrate 1-like [KO:K04392]
108307873  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
108315084  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
108281738  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
108314929  SPG11; spatacsin [KO:K19026]
108296938  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
108318563  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
108291170  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
108299682  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
108303844  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
108288370  UBQLN3; ubiquilin-3 isoform X2 [KO:K04523]
108289197  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
108290218  UBQLN4; ubiquilin-4 [KO:K04523]
108294875  UBQLN2; ubiquilin-2 [KO:K04523]
108314228  transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
108305954  DERL1; derlin-1 isoform X1 [KO:K11519]
108285593  proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
108306058  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
108309687  proteasome subunit alpha type-2-like [KO:K02726] [EC:3.4.25.1]
108292868  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
108296768  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
108298796  proteasome subunit alpha type-4-like [KO:K02728] [EC:3.4.25.1]
108282361  PSMA8; proteasome subunit alpha-type 8 isoform X2 [KO:K02731] [EC:3.4.25.1]
108285946  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
108283109  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
108302169  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
108306111  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
108281267  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
108317705  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
108284787  proteasome subunit beta type-7-like [KO:K02739] [EC:3.4.25.1]
108281590  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
108284382  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
108304855  proteasome subunit beta type-5-like [KO:K02737] [EC:3.4.25.1]
108315265  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
108295247  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
108316429  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
108300288  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
108298560  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 7-like [KO:K03061]
108298258  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
108301412  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
108285679  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
108291927  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
108304839  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
108318267  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
108311077  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
108304748  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
108281507  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
108315147  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
108301820  26S proteasome non-ATPase regulatory subunit 12-like [KO:K03035]
108314101  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
108313687  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
108303512  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
108311781  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
108298295  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
108282790  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 4-like [KO:K03029]
108316441  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
108317417  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
108287895  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
108285952  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
108301852  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
108300901  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
108286284  TAR DNA-binding protein 43 [KO:K23600]
108293912  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
108296474  heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
108317831  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108298281  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108284908  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
108288839  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108282182  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108290680  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108291264  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108312027  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
108313760  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108285065  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108317606  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
108315753  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108296409  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108307326  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108301191  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
108290413  heterogeneous nuclear ribonucleoproteins A2/B1-like [KO:K13158]
108303240  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
108302204  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
108313775  ATXN2; ataxin-2 isoform X1 [KO:K23625]
108310059  MATR3; matrin-3 isoform X1 [KO:K13213]
108303899  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
108293233  THO complex subunit 4-like [KO:K12881]
108301653  ALYREF; THO complex subunit 4 [KO:K12881]
108299991  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
108293757  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
108297537  nuclear RNA export factor 2-like [KO:K14284]
108286198  NXF1; nuclear RNA export factor 1 [KO:K14284]
108295756  nuclear RNA export factor 2-like [KO:K14284]
108295772  NXF3; nuclear RNA export factor 3 isoform X1 [KO:K14284]
108313163  NXT1; NTF2-related export protein 1 [KO:K14285]
108316557  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
108318156  GLE1; nucleoporin GLE1 [KO:K18723]
108301376  TPR; nucleoprotein TPR [KO:K09291]
108304987  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
108298923  NUP153; nuclear pore complex protein Nup153 [KO:K14296]
108307506  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
108309957  RAE1; mRNA export factor [KO:K14298]
108284658  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
108282480  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
108302766  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
108300141  NUP107; nuclear pore complex protein Nup107 [KO:K14301]
108294825  NUP37; nucleoporin Nup37 [KO:K14302]
108318403  NUP160; nuclear pore complex protein Nup160 [KO:K14303]
108309408  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
108287380  NUP43; nucleoporin Nup43 [KO:K14305]
108292016  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
108316539  NUP62CL; nucleoporin-62 C-terminal-like protein [KO:K14306]
108310489  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
108293431  NUP54; nucleoporin p54 isoform X2 [KO:K14308]
108285477  NUP93; nuclear pore complex protein Nup93 [KO:K14309]
108307217  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
108283028  NUP188; nucleoporin NUP188 homolog [KO:K14311]
108317615  NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
108307374  NUP35; nucleoporin NUP35 isoform X1 [KO:K14313]
108292497  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
108286907  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
108286530  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
108307911  nuclear envelope pore membrane protein POM 121C [KO:K14316]
108297825  POM121L2; POM121-like protein 2 [KO:K14316]
108282987  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
108297769  NUP88; nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
108312957  RANBP2; E3 SUMO-protein ligase RanBP2 [KO:K12172] [EC:2.3.2.-]
108297013  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
108282235  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
108318239  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
108309943  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
108293602  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
108309731  DDIT3; LOW QUALITY PROTEIN: DNA damage-inducible transcript 3 protein [KO:K04452]
108295573  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108283240  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
108287258  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
108305646  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108310018  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
119468660  eukaryotic translation initiation factor 2 subunit 1-like [KO:K03237]
108284112  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
108293817  cyclic AMP-dependent transcription factor ATF-4-like [KO:K04374]
108315292  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
108300031  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X3 [KO:K04960]
108314213  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
108299857  TNF; tumor necrosis factor [KO:K03156]
108316345  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
108286567  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
108300048  DAXX; death domain-associated protein 6 [KO:K02308]
108290685  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
108281677  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
108300002  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
108300003  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
108286818  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
108286819  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
108305039  NOS1; nitric oxide synthase, brain isoform X1 [KO:K13240] [EC:1.14.13.39]
108310157  CCS; copper chaperone for superoxide dismutase [KO:K04569]
108308252  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
108317957  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
108318060  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
108293540  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
108306748  glutathione peroxidase 2-like [KO:K00432] [EC:1.11.1.9]
108287157  GPX1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
108296059  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
108296091  GPX6; glutathione peroxidase 6 isoform X1 [KO:K00432] [EC:1.11.1.9]
108305771  CAT; catalase [KO:K03781] [EC:1.11.1.6]
108316045  PRPH; peripherin isoform X2 [KO:K07607]
108318590  NEFL; neurofilament light polypeptide [KO:K04572]
108318587  NEFM; neurofilament medium polypeptide isoform X1 [KO:K04573]
108303587  LOW QUALITY PROTEIN: neurofilament heavy polypeptide-like [KO:K04574]
108283352  NEFH; neurofilament heavy polypeptide isoform X2 [KO:K04574]
108300161  PFN2; profilin-2 isoform X1 [KO:K05759]
108291683  profilin-2-like [KO:K05759]
108281256  PFN1; profilin-1 [KO:K05759]
108299017  profilin-1-like [KO:K05759]
108303062  PFN4; profilin-4 [KO:K05759]
108292161  PFN3; profilin-3 [KO:K05759]
108308136  ACTB; actin, cytoplasmic 1 [KO:K05692]
108290515  actin, muscle [KO:K05692]
108301684  ACTG1; actin, cytoplasmic 2 [KO:K05692]
108314985  actin, cytoplasmic 1-like [KO:K05692]
108296942  ANXA7; annexin A7 isoform X1 [KO:K17095]
108281503  ANXA11; annexin A11 isoform X1 [KO:K17095]
108309732  KIF5A; kinesin heavy chain [KO:K10396]
108300456  KIF5C; kinesin heavy chain [KO:K10396]
108309139  KIF5B; kinesin-1 heavy chain [KO:K10396]
108310222  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
108285344  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
108287156  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
108317118  KLC4; kinesin light chain 4 [KO:K10407]
108298147  tubulin alpha-3 chain [KO:K07374]
108298324  tubulin alpha-3 chain [KO:K07374]
108309093  TUBA4A; tubulin alpha-4A chain [KO:K07374]
108292358  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
108281799  tubulin alpha chain-like [KO:K07374]
108290633  tubulin alpha-1A chain-like [KO:K07374]
108302307  tubulin alpha-1B chain-like isoform X2 [KO:K07374]
108283506  tubulin alpha chain [KO:K07374]
119472242  tubulin alpha-4A chain-like [KO:K07374]
108316033  LOW QUALITY PROTEIN: tubulin alpha-1C chain [KO:K07374]
108294861  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
108315998  tubulin alpha-1A chain-like [KO:K07374]
108316038  tubulin alpha-1A chain-like [KO:K07374]
108306955  tubulin alpha-1B chain-like [KO:K07374]
108299116  tubulin beta chain-like [KO:K07375]
108292848  LOW QUALITY PROTEIN: tubulin beta-3 chain [KO:K07375]
108281778  TUBB2A; tubulin beta-2A chain [KO:K07375]
108290576  TUBB; tubulin beta chain [KO:K07375]
108297202  tubulin beta chain-like [KO:K07375]
108298289  tubulin beta-8 chain-like [KO:K07375]
108282495  TUBB6; tubulin beta-6 chain isoform X2 [KO:K07375]
108304222  TUBB4A; tubulin beta-4A chain [KO:K07375]
108285924  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
108294959  tubulin beta-8 chain-like [KO:K07375]
108316531  tubulin beta-8 chain-like [KO:K07375]
108287238  TUBB4B; tubulin beta-4B chain [KO:K07375]
108289303  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
108309730  DCTN2; dynactin subunit 2 isoform X2 [KO:K10424]
108314206  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
108293543  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
108291812  LOW QUALITY PROTEIN: dynactin subunit 5-like [KO:K10427]
108308396  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
108309453  DCTN6; dynactin subunit 6 [KO:K10428]
108311612  dynactin subunit 6-like [KO:K10428]
108301037  ACTR1B; beta-centractin isoform X2 [KO:K16575]
108306310  ACTR1A; alpha-centractin [KO:K16575]
108306218  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
108289852  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
108308491  DNAH3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
108293514  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
108301554  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
108301744  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
108311323  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
108302720  DNAH14; dynein heavy chain 14, axonemal isoform X1 [KO:K10408]
108303501  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
108303194  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
108313894  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
108315117  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
108293744  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
108306433  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
108306466  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
108290061  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
108314195  DNAI1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409]
108309362  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
108290074  LOW QUALITY PROTEIN: dynein light chain 1, axonemal-like [KO:K10411]
108304486  DNAL1; dynein light chain 1, axonemal [KO:K10411]
108289522  DNAL4; dynein light chain 4, axonemal [KO:K10412]
108305728  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
108296912  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
108312558  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
108302423  GRIA2; glutamate receptor 2 isoform X3 [KO:K05198]
108287252  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
108308002  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
108304131  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
108309377  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X3 [KO:K05211]
108283875  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
108308927  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X2 [KO:K05455]
108310076  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
108286864  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
108295885  NRG4; pro-neuregulin-4, membrane-bound isoform [KO:K05458]
108308938  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
cimi00190  Oxidative phosphorylation
cimi03013  Nucleocytoplasmic transport
cimi03050  Proteasome
cimi04010  MAPK signaling pathway
cimi04012  ErbB signaling pathway
cimi04020  Calcium signaling pathway
cimi04115  p53 signaling pathway
cimi04137  Mitophagy - animal
cimi04140  Autophagy - animal
cimi04141  Protein processing in endoplasmic reticulum
cimi04210  Apoptosis
cimi04724  Glutamatergic synapse
cimi04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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