KEGG   PATHWAY: pteh05014
Entry
pteh05014                   Pathway                                
Name
Amyotrophic lateral sclerosis - Piliocolobus tephrosceles (Ugandan red Colobus)
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
pteh05014  Amyotrophic lateral sclerosis
pteh05014

Organism
Piliocolobus tephrosceles (Ugandan red Colobus) [GN:pteh]
Gene
111527100  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
111540082  CASP1; caspase-1 [KO:K01370] [EC:3.4.22.36]
113222446  CARD16; caspase recruitment domain-containing protein 16 isoform X1 [KO:K01370] [EC:3.4.22.36]
111527587  BID; BH3-interacting domain death agonist isoform X1 [KO:K04726]
111528437  BCL2; apoptosis regulator Bcl-2 isoform X1 [KO:K02161]
111548123  BCL2L1; bcl-2-like protein 1 isoform X1 [KO:K04570]
111520083  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
111522294  BAD; bcl2-associated agonist of cell death [KO:K02158]
111520510  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
111528103  cytochrome c-like [KO:K08738]
111541425  cytochrome c [KO:K08738]
111551227  cytochrome c-like [KO:K08738]
111551298  cytochrome c-like [KO:K08738]
111551797  cytochrome c [KO:K08738]
111551867  cytochrome c [KO:K08738]
111543337  cytochrome c-like [KO:K08738]
111543628  cytochrome c-like [KO:K08738]
111534452  cytochrome c-like [KO:K08738]
111545049  cytochrome c-like [KO:K08738]
111555014  cytochrome c-like [KO:K08738]
111524229  cytochrome c [KO:K08738]
111525268  cytochrome c-like [KO:K08738]
111539464  cytochrome c-like [KO:K08738]
111526887  cytochrome c-like [KO:K08738]
111540111  cytochrome c-like [KO:K08738]
111527287  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
111551382  caspase-3-like isoform X1 [KO:K02187] [EC:3.4.22.56]
111548479  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
111525058  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
111522229  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
111553523  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X4 [KO:K03943] [EC:7.1.1.2]
111534400  uncharacterized protein LOC111534400 [KO:K03944]
111527268  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944]
111550654  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
113222333  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
111528048  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111530496  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111549930  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111551073  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111541817  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
111543027  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111550191  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
111543405  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
111548932  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
111530270  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
111531360  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
111555913  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
111553739  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
111528910  acyl carrier protein, mitochondrial-like [KO:K03955]
111551947  acyl carrier protein, mitochondrial-like [KO:K03955]
111521032  acyl carrier protein, mitochondrial [KO:K03955]
111524218  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
111543582  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
111534727  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
113225083  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
111553196  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
111554285  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
111555537  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111538232  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111521523  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
111554321  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
111547056  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
111528601  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
111541663  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
111543970  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
111536590  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
111536667  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
111528036  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
111521398  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
111537237  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
111554324  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
111538809  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
111550747  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
111524062  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
113220661  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10-like [KO:K03966]
111536395  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
111547586  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X2 [KO:K03934] [EC:7.1.1.2]
111532158  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial-like [KO:K03935] [EC:7.1.1.2]
111543906  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
111544881  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
111541649  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
111529589  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
111533893  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
113219738  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
111546746  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
111526808  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
111522454  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
111551949  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
111552131  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
111548191  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
111540800  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
111527218  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like [KO:K00234] [EC:1.3.5.1]
113223448  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like isoform X1 [KO:K00234] [EC:1.3.5.1]
111552720  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like [KO:K00234] [EC:1.3.5.1]
113223449  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like isoform X1 [KO:K00234] [EC:1.3.5.1]
111547766  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
111521178  succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
111543913  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
111540134  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
111553897  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
111523545  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
111555381  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
111551163  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
111548783  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
111520885  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
111529388  cytochrome b-c1 complex subunit 8 [KO:K00418]
111525372  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
111520664  cytochrome b-c1 complex subunit 10 [KO:K00420]
111553886  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
111548228  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
111529701  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
111528600  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
111542216  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
111537657  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
111525634  cytochrome c oxidase subunit 6A2, mitochondrial isoform X2 [KO:K02266]
111521110  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
111527024  cytochrome c oxidase subunit 6B1 [KO:K02267]
111529926  cytochrome c oxidase subunit 6B1 [KO:K02267]
113219478  cytochrome c oxidase subunit 6C-like [KO:K02268]
111520904  cytochrome c oxidase subunit 6C [KO:K02268]
111534970  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6C-like [KO:K02268]
111554066  cytochrome c oxidase subunit 6C [KO:K02268]
111554207  cytochrome c oxidase subunit 6C-like [KO:K02268]
111524216  cytochrome c oxidase subunit 6C-like [KO:K02268]
111525843  cytochrome c oxidase subunit 6C-like [KO:K02268]
113222372  cytochrome c oxidase subunit 6C-like [KO:K02268]
116418525  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
111524186  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
111547456  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
111550927  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
111536059  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111548777  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
111541635  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111542387  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111531689  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111533949  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111544463  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111554227  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111554579  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111547726  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111539600  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
111522480  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
111538250  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
111539691  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
111541836  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
111554848  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
111526793  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
111555678  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
111556045  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
111541923  ATP5MC2; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
111520690  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
111542754  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
116418593  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
111537284  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
111542440  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
111525460  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
111524700  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
111529272  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
111543911  TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518]
111527894  TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518]
111537868  MCU; calcium uniporter protein, mitochondrial [KO:K20858]
111547732  PINK1; serine/threonine-protein kinase PINK1, mitochondrial [KO:K05688] [EC:2.7.11.1]
111550282  PRKN; E3 ubiquitin-protein ligase parkin isoform X1 [KO:K04556] [EC:2.3.2.31]
111549976  OPTN; optineurin isoform X1 [KO:K19946]
111527434  SQSTM1; sequestosome-1 isoform X1 [KO:K14381]
111553864  MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
111545183  MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435]
111546653  microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
113219740  microtubule-associated proteins 1A/1B light chain 3B [KO:K10435]
111548165  MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435]
111551230  TBK1; serine/threonine-protein kinase TBK1 [KO:K05410] [EC:2.7.11.10]
111539247  serine/threonine-protein kinase TBK1-like [KO:K05410] [EC:2.7.11.10]
111546076  TANK; TRAF family member-associated NF-kappa-B activator [KO:K12650]
111544412  WDR41; WD repeat-containing protein 41 isoform X1 [KO:K23610]
111526162  SMCR8; guanine nucleotide exchange protein SMCR8 [KO:K23611]
111537226  C9orf72; guanine nucleotide exchange C9orf72 homolog isoform X1 [KO:K23609]
111554270  RAB8A; ras-related protein Rab-8A [KO:K07901]
111527402  RAB39B; ras-related protein Rab-39B [KO:K07925]
111550586  RAB1A; ras-related protein Rab-1A [KO:K07874]
111526309  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
111529825  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
111527821  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
111541978  ATG101; autophagy-related protein 101 [KO:K19730]
111544906  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
111521071  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
111544908  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
111545301  BECN2; beclin-2 [KO:K08334]
111537346  BECN1; beclin-1 isoform X1 [KO:K08334]
111539511  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
111555244  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
111537958  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
111539704  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
111522321  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
111538276  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
111530604  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
111542492  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
111547330  ALS2; alsin isoform X1 [KO:K04575]
111539482  RAB5A; ras-related protein Rab-5A [KO:K07887]
111530500  RAC1; ras-related C3 botulinum toxin substrate 1 [KO:K04392]
111545138  CHMP2B; charged multivesicular body protein 2b isoform X1 [KO:K12192]
111538564  FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913] [EC:3.1.3.-]
111529497  spatacsin isoform X1 [KO:K19026]
113219856  spatacsin isoform X1 [KO:K19026]
111545684  PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111537850  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111547968  PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348] [EC:3.1.3.16]
111550575  PPP3R1; calcineurin subunit B type 1 isoform X1 [KO:K06268]
111536216  PPP3R2; calcineurin subunit B type 2 [KO:K06268]
111540937  UBQLN3; ubiquilin-3 [KO:K04523]
111543387  UBQLN1; ubiquilin-1 isoform X1 [KO:K04523]
111543777  UBQLN4; ubiquilin-4 [KO:K04523]
111535902  UBQLN2; ubiquilin-2 [KO:K04523]
111536776  VCP; transitional endoplasmic reticulum ATPase isoform X3 [KO:K13525]
111550883  DERL1; derlin-1 isoform X1 [KO:K11519]
111520434  proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
111524462  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
111552257  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
111528843  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
111520532  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
111539619  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
111556019  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
111555087  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
111555374  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
111521313  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
111545945  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
111537492  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
111555851  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
111554779  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
111530909  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
111543652  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
111526707  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
111538209  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
111554053  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
111520792  26S proteasome regulatory subunit 10B-like [KO:K03064]
111549444  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
111544887  26S proteasome regulatory subunit 6A [KO:K03065]
111527608  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
111523157  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
111527536  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
111537460  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
111538059  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
111542501  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
111520260  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
111533818  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
111541415  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
111520567  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
111543645  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
111546075  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
111554023  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
111520460  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
111535567  LOW QUALITY PROTEIN: proteasomal ubiquitin receptor ADRM1-like [KO:K06691]
111551074  SEM1; 26S proteasome complex subunit SEM1 isoform X2 [KO:K10881]
111535713  HDAC6; histone deacetylase 6 isoform X1 [KO:K11407] [EC:3.5.1.98]
111526306  TARDBP; TAR DNA-binding protein 43 [KO:K23600]
111525624  FUS; RNA-binding protein FUS isoform X1 [KO:K13098]
111528140  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111550673  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111541909  HNRNPA1; heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
111532953  LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111529855  heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
113225015  heterogeneous nuclear ribonucleoprotein A3-like isoform X1 [KO:K12741]
111537937  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111547744  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111531463  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111543484  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111554494  heterogeneous nuclear ribonucleoprotein A3-like isoform X1 [KO:K12741]
111555572  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111537209  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111546980  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
113220247  heterogeneous nuclear ribonucleoprotein A1 [KO:K12741]
111548784  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111542765  heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741]
111540354  heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741]
111551855  HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158]
111536875  ATXN2; ataxin-2 isoform X1 [KO:K23625]
111526928  ATXN2L; ataxin-2-like protein isoform X1 [KO:K23625]
111528507  MATR3; matrin-3 isoform X1 [KO:K13213]
111522544  ANG; angiogenin [KO:K16631] [EC:3.1.27.-]
111536652  NCBP1; nuclear cap-binding protein subunit 1 isoform X1 [KO:K12882]
111542290  ALYREF; THO complex subunit 4 [KO:K12881]
111542981  SRSF3; serine/arginine-rich splicing factor 3 [KO:K12892]
111545362  SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896]
111530207  nuclear RNA export factor 2 isoform X1 [KO:K14284]
111522301  NXF1; nuclear RNA export factor 1 [KO:K14284]
111535994  LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284]
111535980  NXF3; nuclear RNA export factor 3 [KO:K14284]
111536668  NXT2; NTF2-related export protein 2 isoform X1 [KO:K14285]
111526253  NXT1; NTF2-related export protein 1 [KO:K14285]
111545809  GLE1; nucleoporin GLE1 [KO:K18723]
111536922  TPR; nucleoprotein TPR [KO:K09291]
111548907  NUP50; nuclear pore complex protein Nup50 [KO:K14295]
111541126  NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296]
111540908  NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297]
111555661  RAE1; mRNA export factor [KO:K14298]
111546928  SEC13; protein SEC13 homolog isoform X1 [KO:K14004]
111553512  SEH1L; nucleoporin SEH1 isoform X1 [KO:K14299]
111526205  NUP133; nuclear pore complex protein Nup133 [KO:K14300]
111528619  NUP107; nuclear pore complex protein Nup107 isoform X1 [KO:K14301]
111523600  NUP37; nucleoporin Nup37 isoform X1 [KO:K14302]
111544879  NUP160; nuclear pore complex protein Nup160 [KO:K14303]
111542434  NUP85; nuclear pore complex protein Nup85 isoform X1 [KO:K14304]
111523644  NUP43; nucleoporin Nup43 [KO:K14305]
111520176  NUP62; nuclear pore glycoprotein p62 [KO:K14306]
111554361  nuclear pore glycoprotein p62 [KO:K14306]
111524994  NUP58; nucleoporin p58/p45 isoform X1 [KO:K14307]
111549343  NUP54; nucleoporin p54 [KO:K14308]
111541275  NUP93; nuclear pore complex protein Nup93 isoform X1 [KO:K14309]
111524761  NUP205; nuclear pore complex protein Nup205 [KO:K14310]
111545862  NUP188; nucleoporin NUP188 homolog isoform X2 [KO:K14311]
111545509  NUP155; nuclear pore complex protein Nup155 [KO:K14312]
111542799  NUP35; nucleoporin NUP35 [KO:K14313]
111543940  NUP210L; nuclear pore membrane glycoprotein 210-like [KO:K14314]
111526777  NUP210; nuclear pore membrane glycoprotein 210 isoform X1 [KO:K14314]
111523536  NDC1; nucleoporin NDC1 isoform X1 [KO:K14315]
111547059  nuclear envelope pore membrane protein POM 121C [KO:K14316]
111525903  POM121L2; POM121-like protein 2 [KO:K14316]
111545832  NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317]
111521254  NUP88; nuclear pore complex protein Nup88 [KO:K14318]
111525057  E3 SUMO-protein ligase RanBP2 [KO:K12172] [EC:2.3.2.-]
111535056  SETX; probable helicase senataxin isoform X1 [KO:K10706] [EC:5.6.2.5 5.6.2.3]
111522027  CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial isoform X1 [KO:K22759]
111545930  HSPA5; endoplasmic reticulum chaperone BiP [KO:K09490] [EC:3.6.4.10]
111555642  VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707]
111525029  ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054]
111541810  DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452]
111542532  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
111525377  XBP1; LOW QUALITY PROTEIN: X-box-binding protein 1 [KO:K09027]
111553030  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
111525521  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
111540056  LOW QUALITY PROTEIN: caspase-12-like [KO:K04741] [EC:3.4.22.-]
111528197  EIF2AK3; eukaryotic translation initiation factor 2-alpha kinase 3 isoform X1 [KO:K08860] [EC:2.7.11.1]
111519917  EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237]
111548140  cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
111548991  ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374]
111543148  ITPR3; inositol 1,4,5-trisphosphate receptor type 3 [KO:K04960]
111536786  SIGMAR1; sigma non-opioid intracellular receptor 1 isoform X1 [KO:K20719]
111523940  TNF; tumor necrosis factor [KO:K03156]
111555942  TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158]
111526326  TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141]
111542921  death domain-associated protein 6 isoform X1 [KO:K02308]
116418805  DAXX; death domain-associated protein 6 isoform X1 [KO:K02308]
111528301  MAP2K3; dual specificity mitogen-activated protein kinase kinase 3 isoform X1 [KO:K04432] [EC:2.7.12.2]
111542478  MAP2K6; dual specificity mitogen-activated protein kinase kinase 6 isoform X1 [KO:K04433] [EC:2.7.12.2]
111542972  MAPK14; mitogen-activated protein kinase 14 isoform X1 [KO:K04441] [EC:2.7.11.24]
111542973  MAPK13; mitogen-activated protein kinase 13 isoform X1 [KO:K04441] [EC:2.7.11.24]
111532954  MAPK12; mitogen-activated protein kinase 12 [KO:K04441] [EC:2.7.11.24]
111549140  MAPK11; mitogen-activated protein kinase 11 isoform X1 [KO:K04441] [EC:2.7.11.24]
111537519  NOS1; nitric oxide synthase, brain isoform X1 [KO:K13240] [EC:1.14.13.39]
111522366  CCS; copper chaperone for superoxide dismutase [KO:K04569]
111541637  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
111552657  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
111523690  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
111555592  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
111555941  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
111525985  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
111525999  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
111521868  CAT; catalase [KO:K03781] [EC:1.11.1.6]
111541714  PRPH; peripherin [KO:K07607]
111545716  NEFL; neurofilament light polypeptide [KO:K04572]
111545715  NEFM; neurofilament medium polypeptide isoform X1 [KO:K04573]
111525390  NEFH; LOW QUALITY PROTEIN: neurofilament heavy polypeptide [KO:K04574]
111521348  PFN1; profilin-1 [KO:K05759]
111521598  PFN3; profilin-3 [KO:K05759]
111544740  PFN2; profilin-2 isoform X1 [KO:K05759]
111554439  PFN4; profilin-4 [KO:K05759]
111552124  actin, cytoplasmic 2 [KO:K05692]
111530610  ACTB; actin, cytoplasmic 1 [KO:K05692]
113225257  actin, cytoplasmic 2 [KO:K05692]
111533492  actin, cytoplasmic 1 [KO:K05692]
111546247  actin, cytoplasmic 1-like [KO:K05692]
111526135  actin, cytoplasmic 2-like [KO:K05692]
111542306  ACTG1; actin, cytoplasmic 2 [KO:K05692]
111537798  ANXA11; annexin A11 isoform X1 [KO:K17095]
111537853  ANXA7; annexin A7 isoform X1 [KO:K17095]
111541800  KIF5A; kinesin heavy chain [KO:K10396]
111546128  KIF5C; kinesin heavy chain [KO:K10396]
111546687  KIF5B; kinesin-1 heavy chain [KO:K10396]
111520077  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
111543063  KLC4; kinesin light chain 4 [KO:K10407]
111522459  KLC2; kinesin light chain 2 [KO:K10407]
111538326  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
113219590  tubulin alpha-3 chain [KO:K07374]
113223361  tubulin alpha-3 chain [KO:K07374]
113219534  tubulin alpha-3 chain [KO:K07374]
111528325  tubulin alpha-3 chain-like [KO:K07374]
111541532  tubulin alpha-3 chain [KO:K07374]
111529485  tubulin alpha-1A chain [KO:K07374]
111542034  tubulin alpha-1A chain [KO:K07374]
111542035  tubulin alpha-1C chain [KO:K07374]
111542037  TUBA1B; tubulin alpha-1B chain [KO:K07374]
111530477  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
111554861  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
111548887  TUBA4A; tubulin alpha-4A chain [KO:K07374]
111530753  tubulin beta-8 chain-like isoform X1 [KO:K07375]
113225258  tubulin beta-2A chain [KO:K07375]
111532042  tubulin beta-4B chain [KO:K07375]
111553553  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
111553873  tubulin beta-3 chain isoform X1 [KO:K07375]
111522660  TUBB4A; tubulin beta-4A chain [KO:K07375]
111536045  tubulin beta-4A chain-like [KO:K07375]
111555679  TUBB1; tubulin beta-1 chain [KO:K07375]
111548566  tubulin beta-2B chain [KO:K07375]
111527851  TUBB; tubulin beta chain [KO:K07375]
111533296  tubulin beta-8 chain [KO:K07375]
111523453  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
111541809  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
111536789  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
111552697  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
111524211  DCTN5; dynactin subunit 5 [KO:K10427]
111544159  DCTN6; dynactin subunit 6 [KO:K10428]
111542214  ACTR1B; beta-centractin isoform X1 [KO:K16575]
111538847  ACTR1A; alpha-centractin [KO:K16575]
111554850  ACTR10; actin-related protein 10 [KO:K16576]
111537699  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
111528783  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
111551166  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
111546765  DNAH5; dynein heavy chain 5, axonemal isoform X1 [KO:K10408]
111542546  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
111523467  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
111550609  DNAH10; dynein heavy chain 10, axonemal isoform X2 [KO:K10408]
111527065  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
116418713  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
111537031  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
111545080  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
111546466  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
111543155  DNAH8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
111529573  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
111536841  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
111542580  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
111520574  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
111549004  DNAL4; dynein light chain 4, axonemal [KO:K10412]
111521877  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
111520811  NOS2; nitric oxide synthase, inducible [KO:K13241] [EC:1.14.13.39]
111552681  GRIA1; glutamate receptor 1 [KO:K05197]
111553388  GRIA2; glutamate receptor 2 isoform X2 [KO:K05198]
111553102  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
111551718  GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209]
111552106  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
111542444  GRIN2C; glutamate receptor ionotropic, NMDA 2C [KO:K05211]
111520094  GRIN2D; glutamate receptor ionotropic, NMDA 2D [KO:K05212]
111544148  NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455]
111528367  pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
113224859  pro-neuregulin-2, membrane-bound isoform isoform X1 [KO:K05456]
111551984  NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457]
111530419  NRG4; pro-neuregulin-4, membrane-bound isoform [KO:K05458]
111548039  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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Ultrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesis.
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Front Cell Neurosci 9:341 (2015)
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Reference
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Menzies FM, Fleming A, Rubinsztein DC
  Title
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Nat Rev Neurosci 16:345-57 (2015)
DOI:10.1038/nrn3961
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Pathomechanism Heterogeneity in the Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disease Spectrum: Providing Focus Through the Lens of Autophagy.
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J Mol Biol 432:2692-2713 (2020)
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Curr Top Dev Biol 121:111-171 (2017)
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Nat Rev Neurosci 15:233-49 (2014)
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J Cell Mol Med 15:2025-39 (2011)
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Curr Mol Med 6:79-86 (2006)
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ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
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Genes Dev 22:1451-64 (2008)
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IUBMB Life 63:754-63 (2011)
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Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
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Acta Neuropathol Commun 7:103 (2019)
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Trends Genet 34:404-423 (2018)
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Front Mol Neurosci 7:63 (2014)
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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.
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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
pteh00190  Oxidative phosphorylation
pteh03013  Nucleocytoplasmic transport
pteh03050  Proteasome
pteh04010  MAPK signaling pathway
pteh04012  ErbB signaling pathway
pteh04020  Calcium signaling pathway
pteh04115  p53 signaling pathway
pteh04137  Mitophagy - animal
pteh04140  Autophagy - animal
pteh04141  Protein processing in endoplasmic reticulum
pteh04210  Apoptosis
pteh04724  Glutamatergic synapse
pteh04810  Regulation of actin cytoskeleton
KO pathway
ko05014   
LinkDB

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