KEGG   PATHWAY: rbb05014
Entry
rbb05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Rhinopithecus bieti (black snub-nosed monkey)
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
rbb05014  Amyotrophic lateral sclerosis
rbb05014

Organism
Rhinopithecus bieti (black snub-nosed monkey) [GN:rbb]
Gene
108514363  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
108513176  caspase-1-like [KO:K01370] [EC:3.4.22.36]
108513177  caspase-1-like [KO:K01370] [EC:3.4.22.36]
108513179  CASP1; caspase-1 [KO:K01370] [EC:3.4.22.36]
108541711  BID; BH3-interacting domain death agonist [KO:K04726]
108526412  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
108524131  BCL2L1; bcl-2-like protein 1 isoform X1 [KO:K04570]
108523624  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
108542734  BAD; bcl2-associated agonist of cell death isoform X1 [KO:K02158]
108543462  APAF1; LOW QUALITY PROTEIN: apoptotic protease-activating factor 1 [KO:K02084]
108530087  cytochrome c-like [KO:K08738]
108532915  cytochrome c [KO:K08738]
108535521  cytochrome c [KO:K08738]
108524009  cytochrome c [KO:K08738]
108538827  cytochrome c-like [KO:K08738]
108536287  cytochrome c-like [KO:K08738]
108524923  cytochrome c-like [KO:K08738]
108523439  cytochrome c-like [KO:K08738]
108533297  cytochrome c-like [KO:K08738]
108524269  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
108512689  LOW QUALITY PROTEIN: caspase-3-like [KO:K02187] [EC:3.4.22.56]
108536840  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
108538089  LOW QUALITY PROTEIN: superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
10549380  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
10549368  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
10549374  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
10549376  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
10549375  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
10549377  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
10549378  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
108518679  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
108525115  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
108517346  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
108536327  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
108526355  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
108541984  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
108529459  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947]
108519700  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
108530843  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
108512012  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
108530974  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
108515311  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
108543568  NDUFA6; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
108534515  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
108537102  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
108536503  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
108532675  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
108531931  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
108538844  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
108526312  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 isoform X1 [KO:K03956]
108521802  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
108515623  NDUFA13; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
108519146  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
108541536  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
108534879  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
108532175  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
108541689  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
108516239  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
108532882  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
108529800  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
108538409  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7-like [KO:K03963]
108514796  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
108537065  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7-like [KO:K03963]
108526074  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7-like [KO:K03963]
108535273  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
108519781  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
108538081  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
108519410  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
108523036  NDUFB11; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
108543433  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
108521164  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
108524673  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
108516683  NDUFS4; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
108518004  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
108515730  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
108512485  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
108542935  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
108514381  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
108522161  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
108530254  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
108518677  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
108522756  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
108543209  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
108515327  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
108521165  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
108517325  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
108530376  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
108536453  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
108532893  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
108530603  cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
108530604  cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
10549379  CYTB; cytochrome b [KO:K00412]
108536542  cytochrome c1, heme protein, mitochondrial [KO:K00413]
108527019  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
108514155  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
108535631  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
108531701  cytochrome b-c1 complex subunit 7 [KO:K00417]
108529577  cytochrome b-c1 complex subunit 8 [KO:K00418]
108517591  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
108514742  cytochrome b-c1 complex subunit 10 [KO:K00420]
108530245  cytochrome b-c1 complex subunit 10 [KO:K00420]
10549373  COX3; cytochrome c oxidase subunit III [KO:K02262]
10549369  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
10549370  COX2; cytochrome c oxidase subunit II [KO:K02261]
108521006  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
108524146  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
108531483  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
108529477  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
108527757  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
108529661  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
108512335  cytochrome c oxidase subunit 6B1 [KO:K02267]
108542547  cytochrome c oxidase subunit 6B2 [KO:K02267]
108536532  cytochrome c oxidase subunit 6C [KO:K02268]
108538160  cytochrome c oxidase subunit 6C [KO:K02268]
108541227  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6C-like [KO:K02268]
108526140  cytochrome c oxidase subunit 6C [KO:K02268]
108543345  cytochrome c oxidase subunit 6C-like [KO:K02268]
108513938  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
108542554  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
108535228  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
108513215  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
108535638  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
108519915  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
108530029  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
108527083  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108530984  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108531248  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108542095  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108541117  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108538456  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108542119  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108518799  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108523175  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108516634  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108540721  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108525816  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108517636  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108519118  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
108525188  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
108513794  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
108519025  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
108516042  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
108529918  ATP5C1; LOW QUALITY PROTEIN: ATP synthase subunit gamma, mitochondrial [KO:K02136]
108530260  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
108541621  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
10549372  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
108528082  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
108514651  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
108527161  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
108516511  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
108537436  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
108536866  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
108518287  ATP5J; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
10549371  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
108529563  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
108521188  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
108531118  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
108516879  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
108515300  PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
108527984  PARK2; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
108531309  OPTN; optineurin [KO:K19946]
108523366  SQSTM1; sequestosome-1 [KO:K14381]
108520768  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
108512512  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
108533773  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
108529966  TBK1; LOW QUALITY PROTEIN: serine/threonine-protein kinase TBK1 [KO:K05410] [EC:2.7.11.10]
108513559  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
108544434  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
108516620  SMCR8; Smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611]
108527848  CUNH9orf72; protein C9orf72 homolog isoform X1 [KO:K23609]
108543281  RAB8A; ras-related protein Rab-8A [KO:K07901]
108540748  RAB39B; ras-related protein Rab-39B [KO:K07925]
108527867  ras-related protein Rab-1A-like [KO:K07874]
108540804  RAB1A; ras-related protein Rab-1A [KO:K07874]
108539366  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
108527503  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
108521945  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
108540897  serine/threonine-protein kinase ULK2-like [KO:K08269] [EC:2.7.11.1]
108521610  ATG101; autophagy-related protein 101 [KO:K19730]
108521955  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
108526927  RB1CC1; LOW QUALITY PROTEIN: RB1-inducible coiled-coil protein 1 [KO:K17589]
108526772  BECN1; beclin-1 isoform X1 [KO:K08334]
108512516  BECN2; beclin-2 [KO:K08334]
108522247  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
108530670  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
108533156  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
108525730  LOW QUALITY PROTEIN: nuclear receptor-binding factor 2-like [KO:K21246]
108537659  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
108519131  ATG2B; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B [KO:K17906]
108543000  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
108530419  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
108523042  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
108513034  ALS2; alsin isoform X1 [KO:K04575]
108521254  RAB5A; ras-related protein Rab-5A [KO:K07887]
108523982  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
108515883  ras-related C3 botulinum toxin substrate 1-like isoform X1 [KO:K04392]
108512778  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
108540544  FIG4; LOW QUALITY PROTEIN: polyphosphoinositide phosphatase [KO:K22913] [EC:3.1.3.-]
108521290  SPG11; spatacsin [KO:K19026]
108542795  serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348] [EC:3.1.3.16]
108516875  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
108540785  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
108537161  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
108529850  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
108536091  UBQLN2; ubiquilin-2 [KO:K04523]
108538969  UBQLN3; ubiquilin-3 [KO:K04523]
108515542  UBQLN4; ubiquilin-4 isoform X1 [KO:K04523]
108524470  VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
108517777  DERL1; derlin-1 [KO:K11519]
108519614  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
108522730  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
108537975  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
108520537  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
108535266  PSMA7; LOW QUALITY PROTEIN: proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
108519637  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
108535520  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
108529724  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
108524844  PSMB6; LOW QUALITY PROTEIN: proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
108518216  proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
108531375  PSMB7; proteasome subunit beta type-7 isoform X1 [KO:K02739] [EC:3.4.25.1]
108533955  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
108537259  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
108523600  PSMB5; LOW QUALITY PROTEIN: proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
108515681  proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
108541286  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
108533229  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
108515543  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
108535903  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
108539058  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
108513654  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
108516314  PSMC3; 26S protease regulatory subunit 6A [KO:K03065]
108536276  PSMC5; 26S protease regulatory subunit 8 isoform X1 [KO:K03066]
108525031  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
108538318  PSMD1; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
108527793  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
108523391  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
108517733  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
108541378  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
108531525  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
108514539  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
108537987  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
108533239  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
108517714  PSMD14; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
108539077  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
108535274  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
108513536  SHFM1; 26S proteasome complex subunit DSS1 isoform X1 [KO:K10881]
108517465  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
108539382  TARDBP; TAR DNA-binding protein 43 [KO:K23600]
108528462  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
108538097  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108514619  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
108521534  heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
108527137  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
108523002  heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
108536466  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108539856  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A3-like [KO:K12741]
108532758  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108513257  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108535575  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108541980  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
108517879  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
108512237  ATXN2; LOW QUALITY PROTEIN: ataxin-2 [KO:K23625]
108523670  ATXN2L; LOW QUALITY PROTEIN: ataxin-2-like protein [KO:K23625]
108544074  MATR3; matrin-3 isoform X1 [KO:K13213]
108527319  ANG; angiogenin [KO:K16631] [EC:3.1.27.-]
108539106  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
108534261  ALYREF; THO complex subunit 4 [KO:K12881]
108528335  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
108520872  SRSF7; serine/arginine-rich splicing factor 7 [KO:K12896]
108540254  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
108545015  NXF3; nuclear RNA export factor 3 [KO:K14284]
108520077  nuclear RNA export factor 2-like [KO:K14284]
108533563  NXF1; nuclear RNA export factor 1 [KO:K14284]
108520665  NXT1; NTF2-related export protein 1 [KO:K14285]
108512802  GLE1; nucleoporin GLE1 isoform X1 [KO:K18723]
108512698  TPR; LOW QUALITY PROTEIN: nucleoprotein TPR [KO:K09291]
108514513  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
108531023  NUP153; LOW QUALITY PROTEIN: nuclear pore complex protein Nup153 [KO:K14296]
108543753  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
108515321  RAE1; mRNA export factor isoform X1 [KO:K14298]
108512292  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
108544555  protein SEC13 homolog [KO:K14004]
108533092  SEH1L; nucleoporin SEH1 [KO:K14299]
108537831  NUP133; LOW QUALITY PROTEIN: nuclear pore complex protein Nup133 [KO:K14300]
108532252  NUP107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
108543132  NUP37; nucleoporin Nup37 isoform X1 [KO:K14302]
108524678  NUP160; nuclear pore complex protein Nup160 isoform X1 [KO:K14303]
108537424  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
108536665  NUP43; nucleoporin Nup43 [KO:K14305]
108514088  nucleoporin Nup43-like [KO:K14305]
108535812  NUP62; nuclear pore glycoprotein p62 isoform X1 [KO:K14306]
108515453  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
108532488  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
108533862  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
108539208  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
108512824  NUP188; nucleoporin NUP188 homolog [KO:K14311]
108515967  NUP155; nuclear pore complex protein Nup155 [KO:K14312]
108526639  NUP35; nucleoporin NUP53 isoform X1 [KO:K14313]
108524785  LOW QUALITY PROTEIN: nuclear pore membrane glycoprotein 210-like [KO:K14314]
108518827  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
108517660  LOW QUALITY PROTEIN: nuclear pore membrane glycoprotein 210-like [KO:K14314]
108531214  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
108520549  POM121L2; POM121-like protein 2 [KO:K14316]
108518936  POM121C; nuclear envelope pore membrane protein POM 121C isoform X1 [KO:K14316]
108513925  NUP214; nuclear pore complex protein Nup214 [KO:K14317]
108514343  NUP88; nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
108536784  LOW QUALITY PROTEIN: E3 SUMO-protein ligase RanBP2-like [KO:K12172] [EC:2.3.2.-]
108513948  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
108540429  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
108531361  HSPA5; 78 kDa glucose-regulated protein [KO:K09490] [EC:3.6.4.10]
108515324  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
108542583  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
108519685  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
108536259  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
108517565  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
108542901  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
108522968  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
108513174  LOW QUALITY PROTEIN: inactive caspase-12-like [KO:K04741] [EC:3.4.22.-]
108518882  EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860] [EC:2.7.11.1]
108533712  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
108520463  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
108516255  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
108542166  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
108520255  TNF; tumor necrosis factor isoform X1 [KO:K03156]
108536511  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
108539384  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
108540273  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
108521513  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
108523215  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
108528326  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
108528327  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
108539403  MAPK12; mitogen-activated protein kinase 12 isoform X1 [KO:K04441] [EC:2.7.11.24]
108539422  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
108539288  NOS1; LOW QUALITY PROTEIN: nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
108518660  CCS; copper chaperone for superoxide dismutase isoform X1 [KO:K04569]
108540124  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
108540127  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
108530977  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
108532034  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
108530299  glutathione peroxidase 1-like isoform X1 [KO:K00432] [EC:1.11.1.9]
108542423  GPX2; glutathione peroxidase 2 isoform X1 [KO:K00432] [EC:1.11.1.9]
108526993  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
108531237  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
108513829  CAT; catalase [KO:K03781] [EC:1.11.1.6]
108543959  PRPH; peripherin isoform X1 [KO:K07607]
108537813  NEFL; neurofilament light polypeptide [KO:K04572]
108520451  LOW QUALITY PROTEIN: neurofilament light polypeptide-like [KO:K04572]
108537822  NEFM; neurofilament medium polypeptide [KO:K04573]
108517592  NEFH; neurofilament heavy polypeptide isoform X1 [KO:K04574]
108518278  PFN2; profilin-2 [KO:K05759]
108544776  PFN3; profilin-3 [KO:K05759]
108524810  PFN1; profilin-1 [KO:K05759]
108523453  profilin-2-like [KO:K05759]
108534125  ACTG1; actin, cytoplasmic 2 [KO:K05692]
108538237  ACTB; actin, cytoplasmic 1 [KO:K05692]
108518085  ANXA11; annexin A11 isoform X1 [KO:K17095]
108516854  ANXA7; annexin A7 isoform X1 [KO:K17095]
108534025  KIF5C; kinesin heavy chain [KO:K10396]
108519708  KIF5A; kinesin heavy chain [KO:K10396]
108515926  KIF5B; LOW QUALITY PROTEIN: kinesin-1 heavy chain [KO:K10396]
108533475  KLC4; kinesin light chain 4 [KO:K10407]
108518649  KLC2; kinesin light chain 2 [KO:K10407]
108531101  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
108521876  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
108541714  TUBA8; tubulin alpha-8 chain [KO:K07374]
108525726  tubulin alpha-1 chain [KO:K07374]
108529896  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
108518420  tubulin alpha-4A chain-like [KO:K07374]
108525461  tubulin alpha-1A chain-like [KO:K07374]
108522550  TUBB; tubulin beta chain [KO:K07375]
108520633  tubulin beta-7 chain [KO:K07375]
108515747  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
108524752  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
108542832  TUBB4B; LOW QUALITY PROTEIN: tubulin beta-4B chain [KO:K07375]
108532059  tubulin beta-4B chain-like [KO:K07375]
108534562  TUBB4A; tubulin beta-4A chain [KO:K07375]
108529080  TUBB3; LOW QUALITY PROTEIN: tubulin beta-3 chain [KO:K07375]
108541619  TUBB1; tubulin beta-1 chain [KO:K07375]
108520672  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
108519684  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
108542163  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
108532078  DCTN4; LOW QUALITY PROTEIN: dynactin subunit 4 [KO:K10426]
108514206  dynactin subunit 4 [KO:K10426]
108538851  DCTN5; dynactin subunit 5 [KO:K10427]
108518591  DCTN6; dynactin subunit 6 [KO:K10428]
108517728  LOW QUALITY PROTEIN: dynactin subunit 6-like [KO:K10428]
108515089  ACTR1A; alpha-centractin [KO:K16575]
108529479  ACTR1B; beta-centractin [KO:K16575]
108529707  ACTR10; LOW QUALITY PROTEIN: actin-related protein 10 [KO:K16576]
108534667  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
108527812  DNAH7; LOW QUALITY PROTEIN: dynein heavy chain 7, axonemal [KO:K10408]
108534178  DNAH17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
108542812  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
108542989  DNAH5; LOW QUALITY PROTEIN: dynein heavy chain 5, axonemal [KO:K10408]
108522346  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
108514126  DNAH3; LOW QUALITY PROTEIN: dynein heavy chain 3, axonemal [KO:K10408]
108523235  LOW QUALITY PROTEIN: dynein heavy chain 7, axonemal-like [KO:K10408]
108528299  DNAH8; LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal [KO:K10408]
108539990  DNAH10; LOW QUALITY PROTEIN: dynein heavy chain 10, axonemal [KO:K10408]
108514365  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
108543583  DNAH12; LOW QUALITY PROTEIN: dynein heavy chain 12, axonemal [KO:K10408]
108517314  DNAH9; LOW QUALITY PROTEIN: dynein heavy chain 9, axonemal [KO:K10408]
108529321  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
108542209  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
108542180  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
108537451  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
108534893  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
108521444  DNAL4; dynein light chain 4, axonemal [KO:K10412]
108513813  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
108539959  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
108532018  GRIA1; LOW QUALITY PROTEIN: glutamate receptor 1 [KO:K05197]
108529017  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
108542864  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
108541249  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
108530279  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
108537403  GRIN2C; glutamate receptor ionotropic, NMDA 2C isoform X1 [KO:K05211]
108523108  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
108518692  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
108530822  NRG2; pro-neuregulin-2, membrane-bound isoform [KO:K05456]
108536716  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
108519185  NRG4; pro-neuregulin-4, membrane-bound isoform [KO:K05458]
108517585  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
rbb00190  Oxidative phosphorylation
rbb03013  Nucleocytoplasmic transport
rbb03050  Proteasome
rbb04010  MAPK signaling pathway
rbb04012  ErbB signaling pathway
rbb04020  Calcium signaling pathway
rbb04115  p53 signaling pathway
rbb04137  Mitophagy - animal
rbb04140  Autophagy - animal
rbb04141  Protein processing in endoplasmic reticulum
rbb04210  Apoptosis
rbb04724  Glutamatergic synapse
rbb04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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