KEGG   PATHWAY: mmf05014
Entry
mmf05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Molossus molossus (Pallas's mastiff bat)
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
mmf05014  Amyotrophic lateral sclerosis
mmf05014

Organism
Molossus molossus (Pallas's mastiff bat) [GN:mmf]
Gene
118634552  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
118627674  caspase-1-like [KO:K01370] [EC:3.4.22.36]
118624690  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
118619009  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
118618234  BCL2L1; bcl-2-like protein 1 [KO:K04570]
118638701  BAX; apoptosis regulator BAX [KO:K02159]
118627989  BAD; bcl2-associated agonist of cell death [KO:K02158]
118623170  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
118620489  cytochrome c [KO:K08738]
118638529  cytochrome c-like [KO:K08738]
118615920  cytochrome c-like [KO:K08738]
118626427  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
118622705  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
118617715  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
118628491  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
118618922  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
118617761  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
118624749  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial-like [KO:K03944]
118636240  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
118615822  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
118636132  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
118639270  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
118641507  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
118620175  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
118620432  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
118622983  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
118624392  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
118617404  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
118619959  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
118620352  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
118628841  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
118624219  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
118643615  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
118629630  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
118623664  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
118625722  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
118641728  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
118625530  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
118615826  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
118623430  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
118616212  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
118635562  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
118639258  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
118620023  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
118625554  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
118617418  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
118617206  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
118629707  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
118615911  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
118632679  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
118631404  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
118642383  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
118635751  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
118625186  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
118637356  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
118627637  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
118642847  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
118627153  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
118636589  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
118642440  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
118616172  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
118628795  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
118616082  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
118628115  NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
118641768  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
118626881  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
118637582  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
118627931  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
118638700  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
118643333  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
118633355  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
118642275  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
118626099  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
118618164  cytochrome b-c1 complex subunit 7-like [KO:K00417]
118631617  cytochrome b-c1 complex subunit 7 [KO:K00417]
118615608  cytochrome b-c1 complex subunit 8 [KO:K00418]
118639942  cytochrome b-c1 complex subunit 9 [KO:K00419]
118615755  cytochrome b-c1 complex subunit 10 [KO:K00420]
118618823  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
118639463  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
118616232  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
118626867  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
118627159  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
118631187  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
118640293  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
118642653  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
118634769  cytochrome c oxidase subunit 6B1-like [KO:K02267]
118620770  cytochrome c oxidase subunit 6B1-like [KO:K02267]
118638658  cytochrome c oxidase subunit 6B1 [KO:K02267]
118638808  cytochrome c oxidase subunit 6B2 [KO:K02267]
118617840  cytochrome c oxidase subunit 6C-like [KO:K02268]
118631656  cytochrome c oxidase subunit 6C [KO:K02268]
118640175  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
118621159  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
118630759  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
118638661  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
118640230  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
118618411  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
118627002  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
118635961  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
118615384  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
118626491  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
118642243  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
118615936  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
118628022  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
118624707  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
118618785  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
118616339  ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
118639201  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
118624196  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
118638769  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
118615754  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
118618728  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
118637863  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
118625818  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
118634232  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
118620224  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
118623869  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
118634324  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
118643520  ATP synthase subunit d, mitochondrial-like [KO:K02138]
118643635  ATP synthase subunit d, mitochondrial-like [KO:K02138]
118622754  ATP synthase subunit d, mitochondrial-like [KO:K02138]
118631376  ATP synthase subunit d, mitochondrial-like [KO:K02138]
118617273  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
118626752  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
118620305  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
118617089  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
118627971  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
118615375  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
118637322  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
118638691  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
118632183  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
118626081  PINK1; serine/threonine-protein kinase PINK1, mitochondrial isoform X1 [KO:K05688] [EC:2.7.11.1]
118621965  PRKN; E3 ubiquitin-protein ligase parkin [KO:K04556] [EC:2.3.2.31]
118625651  OPTN; optineurin isoform X1 [KO:K19946]
118615289  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
118640747  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
118618750  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
118638990  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
118624185  TBK1; serine/threonine-protein kinase TBK1 isoform X1 [KO:K05410] [EC:2.7.11.10]
118625233  TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650]
118642875  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
118641312  SMCR8; guanine nucleotide exchange protein SMCR8 [KO:K23611]
118629786  C9orf72; guanine nucleotide exchange C9orf72 homolog [KO:K23609]
118615433  RAB8A; ras-related protein Rab-8A isoform X1 [KO:K07901]
118636037  RAB39B; ras-related protein Rab-39B [KO:K07925]
118630498  RAB1A; ras-related protein Rab-1A [KO:K07874]
118627075  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
118639772  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
118641236  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
118624521  ATG101; autophagy-related protein 101 [KO:K19730]
118627558  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
118631718  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
118628767  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
118640494  BECN2; beclin-2 [KO:K08334]
118634172  BECN1; beclin-1 [KO:K08334]
118633009  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
118617072  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
118632039  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
118618488  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
118627856  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
118637829  LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B-like [KO:K17906]
118623688  ATG2B; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog B [KO:K17906]
118634300  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
118615399  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
118625249  ALS2; alsin isoform X1 [KO:K04575]
118633623  RAB5A; ras-related protein Rab-5A isoform X1 [KO:K07887]
118616519  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
118617688  CHMP2B; charged multivesicular body protein 2b [KO:K12192]
118621031  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
118631387  SPG11; spatacsin isoform X1 [KO:K19026]
118634685  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118643595  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118632179  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
118630745  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
118628855  ubiquilin-3-like [KO:K04523]
118636618  UBQLN2; ubiquilin-2 [KO:K04523]
118629521  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
118637213  UBQLN4; ubiquilin-4 [KO:K04523]
118629495  transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525]
118631556  DERL1; derlin-1 isoform X1 [KO:K11519]
118636710  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
118619827  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
118640996  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
118618312  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
118618670  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
118619011  proteasome subunit alpha type-5-like [KO:K02729] [EC:3.4.25.1]
118637325  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
118627899  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
118625478  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
118634297  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
118629896  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
118634210  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
118637314  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
118626021  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
118617880  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
118621835  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
118636959  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
118619587  PSMC2; 26S proteasome regulatory subunit 7 isoform X1 [KO:K03061]
118636800  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
118638481  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
118641521  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
118628106  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
118633737  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
118617179  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
118625194  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
118634718  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
118639810  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
118634946  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
118634574  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
118633114  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
118639413  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
118643121  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
118636955  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
118625668  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
118638522  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
118618247  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
118619200  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
118635893  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
118626290  TAR DNA-binding protein 43 isoform X1 [KO:K23600]
118641859  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
118623949  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 isoform X1 [KO:K12741]
118625227  HNRNPA3; heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
118619583  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
118640235  ATXN2; ataxin-2 isoform X1 [KO:K23625]
118642280  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
118643602  MATR3; matrin-3 isoform X1 [KO:K13213]
118617437  ANG; angiogenin [KO:K16631] [EC:3.1.27.-]
118629275  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
118634395  ALYREF; THO complex subunit 4 [KO:K12881]
118620408  serine/arginine-rich splicing factor 3-like [KO:K12892]
118621228  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
118630830  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
118627606  NXF1; nuclear RNA export factor 1 isoform X1 [KO:K14284]
118636154  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
118636161  nuclear RNA export factor 2-like [KO:K14284]
118636162  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
118636163  LOW QUALITY PROTEIN: nuclear RNA export factor 3-like [KO:K14284]
118636165  NXF3; LOW QUALITY PROTEIN: nuclear RNA export factor 3 [KO:K14284]
118636284  nuclear RNA export factor 2-like [KO:K14284]
118618745  NXT1; NTF2-related export protein 1 [KO:K14285]
118636374  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
118629589  GLE1; nucleoporin GLE1 isoform X1 [KO:K18723]
118636939  TPR; nucleoprotein TPR isoform X1 [KO:K09291]
118641140  NUP50; nuclear pore complex protein Nup50 isoform X1 [KO:K14295]
118626108  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
118628235  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
118618794  RAE1; mRNA export factor isoform X1 [KO:K14298]
118633263  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
118618601  SEH1L; LOW QUALITY PROTEIN: nucleoporin SEH1 [KO:K14299]
118640590  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
118624030  NUP107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
118624639  NUP37; nucleoporin Nup37 isoform X1 [KO:K14302]
118629082  NUP160; nuclear pore complex protein Nup160 [KO:K14303]
118634721  NUP85; nuclear pore complex protein Nup85 [KO:K14304]
118621090  NUP43; nucleoporin Nup43 [KO:K14305]
118636179  NUP62CL; LOW QUALITY PROTEIN: nucleoporin-62 C-terminal-like protein [KO:K14306]
118638999  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
118623620  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
118641121  NUP54; nucleoporin p54 isoform X1 [KO:K14308]
118623210  LOW QUALITY PROTEIN: nucleoporin p54-like [KO:K14308]
118639211  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
118619814  NUP205; nuclear pore complex protein Nup205 isoform X1 [KO:K14310]
118629466  NUP188; nucleoporin NUP188 homolog isoform X1 [KO:K14311]
118641857  NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312]
118625498  NUP35; nucleoporin NUP35 isoform X1 [KO:K14313]
118617689  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
118637226  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
118626120  NDC1; nucleoporin NDC1 [KO:K14315]
118641930  POM121C; nuclear envelope pore membrane protein POM 121C [KO:K14316]
118637141  POM121L2; POM121-like protein 2 [KO:K14316]
118629494  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
118634620  NUP88; nuclear pore complex protein Nup88 isoform X1 [KO:K14318]
118627029  RANBP2; E3 SUMO-protein ligase RanBP2 isoform X1 [KO:K12172] [EC:2.3.2.-]
118630221  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
118640116  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial [KO:K22759]
118629806  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
118618771  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
118637539  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054]
118623918  DDIT3; DNA damage-inducible transcript 3 protein [KO:K04452]
118633993  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
118640291  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
118629846  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
118620900  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
118628924  caspase-12-like isoform X1 [KO:K04741] [EC:3.4.22.-]
118626254  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
118619537  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
118623538  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
118621173  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
118630195  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
118621647  TNF; tumor necrosis factor [KO:K03156]
118623904  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
118626273  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B [KO:K05141]
118621904  DAXX; death domain-associated protein 6 [KO:K02308]
118641294  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
118634140  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
118641186  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
118641187  MAPK11; mitogen-activated protein kinase 11 [KO:K04441] [EC:2.7.11.24]
118621004  MAPK13; mitogen-activated protein kinase 13 [KO:K04441] [EC:2.7.11.24]
118621005  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
118639832  NOS1; nitric oxide synthase, brain isoform X1 [KO:K13240] [EC:1.14.13.39]
118628838  CCS; copper chaperone for superoxide dismutase isoform X1 [KO:K04569]
118625800  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
118632938  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
118626772  GPX7; LOW QUALITY PROTEIN: glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
118642533  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
118621721  epididymal secretory glutathione peroxidase-like isoform X1 [KO:K00432] [EC:1.11.1.9]
118616236  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
118627656  CAT; catalase isoform X1 [KO:K03781] [EC:1.11.1.6]
118624014  PRPH; peripherin isoform X1 [KO:K07607]
118616356  NEFL; neurofilament light polypeptide [KO:K04572]
118616518  NEFM; neurofilament medium polypeptide [KO:K04573]
118639846  NEFH; neurofilament heavy polypeptide [KO:K04574]
118617829  PFN2; profilin-2 isoform X1 [KO:K05759]
118633830  PFN1; profilin-1 [KO:K05759]
118631257  PFN4; profilin-4 [KO:K05759]
118616559  PFN3; profilin-3 [KO:K05759]
118635031  ACTG1; actin, cytoplasmic 2 [KO:K05692]
118616016  ACTB; actin, cytoplasmic 1 [KO:K05692]
118632216  ANXA11; annexin A11 isoform X1 [KO:K17095]
118632273  ANXA7; annexin A7 isoform X1 [KO:K17095]
118625741  KIF5C; kinesin heavy chain [KO:K10396]
118624249  KIF5A; kinesin heavy chain [KO:K10396]
118624582  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
118617327  LOW QUALITY PROTEIN: kinesin light chain 1-like [KO:K10407]
118627620  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
118619939  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
118620904  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
118638964  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
118640027  tubulin alpha-3 chain [KO:K07374]
118640348  tubulin alpha-3 chain-like [KO:K07374]
118623162  tubulin alpha-1C chain [KO:K07374]
118623544  TUBA8; tubulin alpha-8 chain [KO:K07374]
118623659  tubulin alpha-1B chain isoform X1 [KO:K07374]
118632190  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
118616912  LOW QUALITY PROTEIN: tubulin alpha chain-like [KO:K07374]
118625290  TUBA4A; tubulin alpha-4A chain [KO:K07374]
118626022  tubulin beta-2B chain [KO:K07375]
118626389  tubulin beta-2B chain-like [KO:K07375]
118618286  TUBB1; tubulin beta-1 chain [KO:K07375]
118618851  TUBB6; tubulin beta-6 chain [KO:K07375]
118621003  TUBB; tubulin beta chain [KO:K07375]
118629269  TUBB4B; tubulin beta-4B chain [KO:K07375]
118629571  tubulin beta-2A chain-like [KO:K07375]
118615549  TUBB4A; tubulin beta-4A chain [KO:K07375]
118639090  TUBB3; tubulin beta-3 chain [KO:K07375]
118630474  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
118623389  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
118629583  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
118615327  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
118642631  DCTN5; dynactin subunit 5 [KO:K10427]
118622642  DCTN6; dynactin subunit 6 [KO:K10428]
118630697  ACTR1B; beta-centractin isoform X1 [KO:K16575]
118632066  ACTR1A; alpha-centractin [KO:K16575]
118616495  ACTR10; actin-related protein 10 [KO:K16576]
118634227  HAP1; huntingtin-associated protein 1 [KO:K04647]
118633038  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
118639917  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
118633303  DNAH1; LOW QUALITY PROTEIN: dynein heavy chain 1, axonemal [KO:K10408]
118640496  dynein heavy chain 14, axonemal-like [KO:K10408]
118633787  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
118640754  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
118633978  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
118641601  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
118619889  DNAH11; LOW QUALITY PROTEIN: dynein heavy chain 11, axonemal [KO:K10408]
118634888  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
118642262  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
118621403  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
118630726  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
118625003  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
118626567  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
118629755  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
118634029  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
118636520  DNAL1; dynein light chain 1, axonemal [KO:K10411]
118623940  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
118627630  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
118634857  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
118615286  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
118615494  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
118629438  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
118641796  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
118623871  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
118634176  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
118639222  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
118622312  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
118615659  NRG2; pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
118632208  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
118625188  ERBB4; receptor tyrosine-protein kinase erbB-4 isoform X1 [KO:K05085] [EC:2.7.10.1]
Compound
C00001  H2O
C00008  ADP
C00025  L-Glutamate
C00027  Hydrogen peroxide
C00039  DNA
C00046  RNA
C00062  L-Arginine
C00070  Copper
C00076  Calcium cation
C00533  Nitric oxide
C00704  Superoxide
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
C16844  Hydroxyl radical
C16845  Peroxynitrite
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Ubiquitin pathways in neurodegenerative disease.
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Front Mol Neurosci 7:63 (2014)
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PMID:29299811
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Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
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Neurochem Res 44:228-233 (2019)
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Benarroch EE
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Sigma-1 receptor and amyotrophic lateral sclerosis.
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Neurology 91:743-747 (2018)
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Nat Rev Neurosci 14:161-76 (2013)
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Related
pathway
mmf00190  Oxidative phosphorylation
mmf03013  Nucleocytoplasmic transport
mmf03050  Proteasome
mmf04010  MAPK signaling pathway
mmf04012  ErbB signaling pathway
mmf04020  Calcium signaling pathway
mmf04115  p53 signaling pathway
mmf04137  Mitophagy - animal
mmf04140  Autophagy - animal
mmf04141  Protein processing in endoplasmic reticulum
mmf04210  Apoptosis
mmf04724  Glutamatergic synapse
mmf04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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