KEGG   PATHWAY: ocu05014
Entry
ocu05014                    Pathway                                
Name
Amyotrophic lateral sclerosis - Oryctolagus cuniculus (rabbit)
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
ocu05014  Amyotrophic lateral sclerosis
ocu05014

Disease
H00058  Amyotrophic lateral sclerosis (ALS)
Organism
Oryctolagus cuniculus (rabbit) [GN:ocu]
Gene
100008594  [KO:K03965]
100008751  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100008764  [KO:K09490] [EC:3.6.4.10]
100008822  [KO:K05692]
100008833  [KO:K13241] [EC:1.14.13.39]
100008840  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100008870  [KO:K10408]
100008875  BCL2L1; bcl-2-like protein 1 [KO:K04570]
100008920  [KO:K05455]
100008939  [KO:K04960]
100009088  TNF; tumor necrosis factor precursor [KO:K03156]
100009243  NOS1; nitric oxide synthase, brain [KO:K13240] [EC:1.14.13.39]
100009258  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
100009272  ACTB; actin, cytoplasmic 1 [KO:K05692]
100009292  TP53; cellular tumor antigen p53 [KO:K04451]
100009313  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100009327  TUBA1B; tubulin alpha-1B chain [KO:K07374]
100009328  [KO:K05085] [EC:2.7.10.1]
100009343  [KO:K04573]
100009423  PPP3CA; protein phosphatase 3 catalytic subunit alpha [KO:K04348] [EC:3.1.3.16]
100009487  PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform [KO:K04348] [EC:3.1.3.16]
100009511  ANXA11; annexin A11 [KO:K17095]
100101588  [KO:K01370] [EC:3.4.22.36]
100101592  [KO:K04399] [EC:3.4.22.62]
100327269  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial precursor [KO:K02263]
100327270  COX6A1; cytochrome c oxidase subunit 6A1, mitochondrial precursor [KO:K02266]
100328584  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
100328585  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
100328669  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
100328673  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100328771  RAB39B; ras-related protein Rab-39B [KO:K07925]
100328815  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100328875  UBQLN3; ubiquilin-3 [KO:K04523]
100337889  [KO:K05209]
100337966  [KO:K00417]
100338005  [KO:K02737] [EC:3.4.25.1]
100338144  [KO:K14307]
100338219  UQCRQ; cytochrome b-c1 complex subunit 8 [KO:K00418]
100338247  [KO:K02729] [EC:3.4.25.1]
100338265  [KO:K16576]
100338267  [KO:K17095]
100338269  [KO:K03035]
100338394  [KO:K14313]
100338439  [KO:K04452]
100338469  [KO:K12172] [EC:2.3.2.-]
100338505  [KO:K12192]
100338517  [KO:K02727] [EC:3.4.25.1]
100338566  [KO:K02731] [EC:3.4.25.1]
100338777  [KO:K14284]
100339009  [KO:K02131]
100339067  [KO:K03037]
100339082  [KO:K03961]
100339093  [KO:K03237]
100339114  [KO:K14300]
100339142  [KO:K12650]
100339187  [KO:K10424]
100339320  [KO:K02308]
100339383  [KO:K04374]
100339424  [KO:K11351]
100339460  [KO:K14316]
100339597  [KO:K10396] [EC:5.6.1.3]
100339628  [KO:K02271]
100339629  [KO:K23600]
100339642  [KO:K10408]
100339709  [KO:K07874]
100339773  [KO:K14284]
100339785  [KO:K13525]
100339862  [KO:K17906]
100339873  [KO:K04392]
100339897  [KO:K03030]
100339936  [KO:K14285]
100339940  [KO:K09027]
100339953  [KO:K04432] [EC:2.7.12.2]
100340058  [KO:K12741]
100340483  [KO:K07374]
100340498  [KO:K04426] [EC:2.7.11.25]
100340527  [KO:K17908]
100340535  [KO:K14284]
100340645  [KO:K02268]
100340735  [KO:K05759]
100340891  [KO:K03781] [EC:1.11.1.6]
100340939  [KO:K07374]
100340965  [KO:K14305]
100341037  [KO:K05692]
100341050  [KO:K08738]
100341099  GPX6; glutathione peroxidase 6 precursor [KO:K00432] [EC:1.11.1.9]
100341175  [KO:K08738]
100341219  [KO:K02266]
100341228  [KO:K20719]
100341245  [KO:K10408]
100341256  [KO:K00237]
100341372  [KO:K20858]
100341458  [KO:K16575]
100341474  [KO:K02266]
100341515  [KO:K08738]
100341571  [KO:K14296]
100341581  [KO:K07375]
100341640  [KO:K04647]
100341658  [KO:K03063]
100341695  [KO:K04441] [EC:2.7.11.24]
100341741  [KO:K10425]
100341772  [KO:K02132]
100341877  [KO:K04523]
100341941  [KO:K03934] [EC:7.1.1.2]
100341993  [KO:K03955]
100342149  [KO:K02738] [EC:3.4.25.1]
100342157  [KO:K14381]
100342159  [KO:K02728] [EC:3.4.25.1]
100342525  [KO:K16575]
100342633  [KO:K13158]
100342678  [KO:K03954]
100342714  [KO:K03938]
100342740  [KO:K10411]
100342837  [KO:K14284]
100342867  [KO:K10428]
100342996  [KO:K02271]
100343073  [KO:K14316]
100343255  [KO:K02273]
100343289  GPX3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
100343444  [KO:K12741]
100343622  [KO:K02732] [EC:3.4.25.1]
100343626  [KO:K14297]
100343627  [KO:K12741]
100343693  [KO:K02128]
100343793  [KO:K02265]
100343824  [KO:K08333] [EC:2.7.11.1]
100343864  [KO:K14304]
100343982  [KO:K00416]
100344129  [KO:K14311]
100344154  [KO:K02131]
100344161  [KO:K04441] [EC:2.7.11.24]
100344221  [KO:K14309]
100344223  [KO:K14310]
100344233  [KO:K03937]
100344371  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
100344446  [KO:K08334]
100344533  [KO:K06268]
100344724  [KO:K23625]
100344965  [KO:K02131]
100345149  [KO:K05456]
100345232  [KO:K03038]
100345268  [KO:K14318]
100345290  [KO:K06693]
100345307  [KO:K03939]
100345319  [KO:K10426]
100345365  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100345587  [KO:K12741]
100345687  [KO:K10408]
100345745  [KO:K02263]
100345761  [KO:K10408]
100345777  [KO:K03960]
100345931  [KO:K04575]
100345955  [KO:K02270]
100346106  [KO:K03158]
100346169  [KO:K00237]
100346411  [KO:K02264]
100346543  [KO:K00432] [EC:1.11.1.9]
100346579  [KO:K14316]
100346609  [KO:K04648]
100346652  [KO:K07374]
100346670  [KO:K02267]
100346739  [KO:K09291]
100346750  [KO:K02128]
100346804  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
100346817  [KO:K03964]
100347089  [KO:K04523]
100347143  [KO:K00415]
100347238  [KO:K14312]
100347288  [KO:K03962]
100347351  [KO:K14284]
100347480  [KO:K12741]
100347545  [KO:K03968]
100347548  [KO:K14303]
100347571  [KO:K00414]
100347748  [KO:K12892]
100347823  [KO:K03948]
100347858  [KO:K07375]
100347864  [KO:K00419]
100347887  MATR3; matrin-3 [KO:K13213]
100347938  [KO:K03952]
100348075  [KO:K07374]
100348082  [KO:K03028]
100348135  [KO:K00411] [EC:7.1.1.8]
100348141  [KO:K10396] [EC:5.6.1.3]
100348217  [KO:K05197]
100348290  [KO:K07375]
100348449  C9orf72; guanine nucleotide exchange factor C9orf72 homolog isoform X1 [KO:K23609]
100348493  [KO:K14314]
100348523  [KO:K02734] [EC:3.4.25.1]
100348563  [KO:K03949]
100348667  [KO:K12741]
100348984  [KO:K02271]
100349090  [KO:K14316]
100349209  [KO:K07374]
100349229  [KO:K14301]
100349288  [KO:K03950]
100349294  [KO:K14004]
100349320  [KO:K03955]
100349412  [KO:K11518]
100349446  [KO:K02739] [EC:3.4.25.1]
100349465  [KO:K10435]
100349506  [KO:K05862]
100349519  [KO:K10435]
100349558  [KO:K17985]
100349573  [KO:K10427]
100349624  [KO:K10409]
100349642  [KO:K03956]
100349785  [KO:K03066]
100349792  [KO:K07375]
100349872  [KO:K05141]
100350027  [KO:K07374]
100350045  [KO:K07375]
100350046  [KO:K07374]
100350064  [KO:K05613]
100350144  [KO:K22759]
100350198  [KO:K05759]
100350226  [KO:K08331]
100350240  [KO:K19730]
100350252  [KO:K07375]
100350412  [KO:K22913] [EC:3.1.3.-]
100350526  [KO:K02131]
100350580  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
100350591  [KO:K03062]
100350614  [KO:K04441] [EC:2.7.11.24]
100350666  [KO:K05688] [EC:2.7.11.1]
100350704  [KO:K03958]
100350714  [KO:K10707]
100350787  [KO:K00411] [EC:7.1.1.8]
100350795  [KO:K23610]
100350803  [KO:K21357] [EC:2.7.11.1]
100350840  [KO:K21246]
100350916  [KO:K03066]
100350923  [KO:K07887]
100350967  [KO:K07374]
100351037  [KO:K08738]
100351044  [KO:K12741]
100351141  [KO:K03946]
100351460  [KO:K18723]
100351492  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100351600  [KO:K10408]
100351682  [KO:K10408]
100351741  [KO:K10408]
100351783  [KO:K02735] [EC:3.4.25.1]
100351795  [KO:K04523]
100351820  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
100351840  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100351898  [KO:K17589]
100351903  [KO:K14285]
100352168  [KO:K03935] [EC:7.1.1.2]
100352330  [KO:K03953]
100352353  [KO:K08334]
100352362  [KO:K13098]
100352363  [KO:K02268]
100352496  [KO:K12741]
100352563  [KO:K02725] [EC:3.4.25.1]
100352600  [KO:K14308]
100352619  [KO:K14316]
100352693  [KO:K10396] [EC:5.6.1.3]
100352718  [KO:K03951]
100352836  [KO:K03061]
100352881  [KO:K17908]
100353082  [KO:K04572]
100353118  [KO:K08269] [EC:2.7.11.1]
100353196  [KO:K19946]
100353266  [KO:K05210]
100353281  [KO:K13158]
100353292  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X3 [KO:K02136]
100353320  [KO:K02271]
100353478  [KO:K07374]
100353514  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100353562  [KO:K23625]
100353595  [KO:K03031]
100353673  [KO:K08738]
100353849  [KO:K10408]
100353930  [KO:K10407]
100353955  [KO:K03064]
100354000  [KO:K03065]
100354074  [KO:K02127]
100354175  [KO:K14302]
100354254  [KO:K02273]
100354306  [KO:K16631] [EC:3.1.27.-]
100354352  [KO:K07374]
100354365  [KO:K14315]
100354468  [KO:K08738]
100354501  [KO:K04569]
100354603  [KO:K03959]
100354630  [KO:K03036]
100354712  [KO:K08334]
100354724  [KO:K08738]
100354735  [KO:K10407]
100354847  [KO:K08860] [EC:2.7.11.1]
100354982  [KO:K11407] [EC:3.5.1.98]
100355002  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100355027  [KO:K11519]
100355084  [KO:K08738]
100355217  [KO:K02726] [EC:3.4.25.1]
100355230  [KO:K04433] [EC:2.7.12.2]
100355248  [KO:K07374]
100355264  [KO:K03039]
100355295  [KO:K02726] [EC:3.4.25.1]
100355349  [KO:K03936] [EC:7.1.1.2]
100355382  GPX7; glutathione peroxidase 7 precursor [KO:K00432] [EC:1.11.1.9]
100355422  [KO:K02267]
100355435  [KO:K14306]
100355529  [KO:K11352]
100355675  [KO:K02159]
100355680  [KO:K03062]
100355984  [KO:K14317]
100355991  [KO:K04726]
100356070  [KO:K05759]
100356164  [KO:K07203] [EC:2.7.11.1]
100356186  [KO:K14314]
100356245  [KO:K04348] [EC:3.1.3.16]
100356308  [KO:K00415]
100356338  [KO:K05211]
100356448  [KO:K00914] [EC:2.7.1.137]
100356512  [KO:K12741]
100356563  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100356593  [KO:K03948]
100356756  [KO:K02270]
100356786  [KO:K02271]
100356812  [KO:K14299]
100356829  [KO:K02730] [EC:3.4.25.1]
100357153  [KO:K05198]
100357157  [KO:K19026]
100357166  [KO:K03033]
100357169  [KO:K14306]
100357177  [KO:K04574]
100357329  [KO:K08738]
100357346  [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100357380  [KO:K05759]
100357449  [KO:K12882]
100357567  [KO:K07374]
100357660  [KO:K02271]
100357822  [KO:K07607]
100357840  [KO:K08738]
100357931  [KO:K17889]
100357982  [KO:K09027]
100358208  [KO:K00419]
100358239  [KO:K02270]
100358390  [KO:K10410]
100358536  [KO:K09054]
100358541  [KO:K05759]
100358562  [KO:K10706] [EC:5.6.2.5 5.6.2.3]
100358587  [KO:K02158]
100358684  [KO:K03943] [EC:7.1.1.2]
100358689  [KO:K07375]
100358771  [KO:K05410] [EC:2.7.11.10]
100358903  [KO:K10408]
100358947  [KO:K12741]
100359017  [KO:K14298]
100359030  [KO:K10412]
100359099  [KO:K14295]
100359118  [KO:K12741]
100359144  [KO:K02127]
103345133  [KO:K05458]
103345363  [KO:K07901]
103345620  [KO:K11407] [EC:3.5.1.98]
103346035  [KO:K04441] [EC:2.7.11.24]
103346157  [KO:K17906]
103346363  [KO:K05212]
103346555  [KO:K11353]
103346589  [KO:K10408]
103346609  [KO:K10408]
103346740  [KO:K03944]
103347262  [KO:K02731] [EC:3.4.25.1]
103347700  [KO:K12896]
103347723  [KO:K08738]
103348383  [KO:K02084]
103348902  [KO:K03032]
103348994  [KO:K03957]
103349397  [KO:K02161]
103349485  [KO:K00420]
103349801  [KO:K02270]
103350026  [KO:K00236]
103350080  [KO:K14298]
103350677  [KO:K04741] [EC:3.4.22.-]
103350693  [KO:K03967]
103351963  [KO:K14284]
108175360  [KO:K00237]
108175381  [KO:K12741]
108175419  [KO:K03948]
108175479  [KO:K02272]
108175623  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
108175836  [KO:K08738]
108176558  [KO:K05458]
108176887  [KO:K02268]
108176933  [KO:K10881]
108177067  [KO:K03945]
108177248  [KO:K10408]
108177262  [KO:K02272]
108177422  [KO:K08738]
108177708  [KO:K02272]
108178067  [KO:K02272]
108178368  [KO:K10881]
108178730  [KO:K08738]
108178908  [KO:K05457]
127483320  [KO:K08738]
127484381  [KO:K02270]
127484606  [KO:K02270]
127484881  [KO:K03957]
127485438  [KO:K14318]
127485451  [KO:K11518]
127485529  [KO:K00420]
127485802  [KO:K12741]
127486372  [KO:K03942] [EC:7.1.1.2]
127486401  [KO:K03966]
127486440  [KO:K03965]
127486867  [KO:K03941] [EC:7.1.1.2]
127486893  [KO:K05208]
127486945  [KO:K00413]
127487016  [KO:K02730] [EC:3.4.25.1]
127487406  [KO:K02133] [EC:7.1.2.2]
127488114  [KO:K03963]
127488641  [KO:K12741]
127488679  [KO:K07375]
127489209  [KO:K02134]
127489662  [KO:K12741]
127489739  [KO:K10407]
127490113  [KO:K14303]
127490235  [KO:K03939]
127490241  [KO:K06691]
127491124  [KO:K10435]
127492394  [KO:K02270]
808221  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
808222  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
808223  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
808224  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
808225  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
808226  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
808227  CYTB; cytochrome b [KO:K00412]
808228  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
808229  COX3; cytochrome c oxidase subunit III [KO:K02262]
808230  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
808231  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
808232  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
808233  COX2; cytochrome c oxidase subunit II [KO:K02261]
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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Related
pathway
ocu00190  Oxidative phosphorylation
ocu03013  Nucleocytoplasmic transport
ocu03050  Proteasome
ocu04010  MAPK signaling pathway
ocu04012  ErbB signaling pathway
ocu04020  Calcium signaling pathway
ocu04115  p53 signaling pathway
ocu04137  Mitophagy - animal
ocu04140  Autophagy - animal
ocu04141  Protein processing in endoplasmic reticulum
ocu04210  Apoptosis
ocu04724  Glutamatergic synapse
ocu04810  Regulation of actin cytoskeleton
KO pathway
ko05014   

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