KEGG   PATHWAY: csyr05016
Entry
csyr05016                   Pathway                                
Name
Huntington disease - Carlito syrichta (Philippine tarsier)
Description
Huntington disease (HD) is an autosomal-dominant neurodegenerative disorder that primarily affects medium spiny striatal neurons (MSN). The symptoms are choreiform, involuntary movements, personality changes and dementia. HD is caused by a CAG repeat expansion in the IT15 gene, which results in a long stretch of polyglutamine (polyQ) close to the amino-terminus of the HD protein huntingtin (Htt). Mutant Htt (mHtt) has effects both in the cytoplasm and in the nucleus. Full-length huntingtin is cleaved by proteases in the cytoplasm, leading to the formation of cytoplasmic and neuritic aggregates. mHtt also alters vesicular transport and recycling, causes cytosolic and mitochondrial Ca2+  overload, triggers endoplasmic reticulum stress through proteasomal dysfunction, and impairs autophagy function, increasing neuronal death susceptibility. N-terminal fragments containing the polyQ strech translocate to the nucleus where they impair transcription and induce neuronal death.
Class
Human Diseases; Neurodegenerative disease
Pathway map
csyr05016  Huntington disease
csyr05016

Organism
Carlito syrichta (Philippine tarsier) [GN:csyr]
Gene
103265266  CLTA; clathrin light chain A isoform X1 [KO:K04644]
103275988  CLTB; clathrin light chain B [KO:K04645]
103269435  CLTCL1; clathrin heavy chain 2 isoform X2 [KO:K04646]
103273412  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
103249851  HIP1; huntingtin-interacting protein 1 [KO:K04559]
103258089  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
103266304  AP2A2; AP-2 complex subunit alpha-2 isoform X2 [KO:K11824]
103276834  AP2B1; AP-2 complex subunit beta [KO:K11825]
103273270  AP2M1; AP-2 complex subunit mu isoform X2 [KO:K11826]
103266428  AP2S1; AP-2 complex subunit sigma [KO:K11827]
103258066  HTT; huntingtin [KO:K04533]
103266735  DCTN1; dynactin subunit 1 isoform X2 [KO:K04648]
103263162  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
103273388  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
103266105  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
103257864  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
103259145  DCTN6; dynactin subunit 6 [KO:K10428]
103251689  ACTR1B; beta-centractin [KO:K16575]
103252560  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
103259026  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
103274512  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
103261454  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
103259689  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
110595630  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
103272927  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
103264641  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
103268211  LOW QUALITY PROTEIN: dynein heavy chain 11, axonemal-like [KO:K10408]
103274289  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
103265790  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
103265883  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
103257252  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
103263993  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
103273431  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
103255303  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
103262619  DNAL1; dynein light chain 1, axonemal isoform X2 [KO:K10411]
103271264  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
103261248  TUBA4A; tubulin alpha-4A chain isoform X1 [KO:K07374]
103261588  tubulin alpha-1 chain-like [KO:K07374]
103262680  TUBA8; tubulin alpha-8 chain [KO:K07374]
103271714  tubulin alpha-3 chain [KO:K07374]
103253635  tubulin alpha-1 chain-like [KO:K07374]
103261884  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
103273447  TUBA1B; tubulin alpha-1B chain isoform X1 [KO:K07374]
103271353  tubulin alpha-3 chain [KO:K07374]
103269656  tubulin beta-4A chain isoform X1 [KO:K07375]
103270010  TUBB1; tubulin beta-1 chain isoform X2 [KO:K07375]
103250978  tubulin beta-4B chain-like isoform X1 [KO:K07375]
103261319  TUBB; tubulin beta chain isoform X1 [KO:K07375]
103253258  tubulin beta-6 chain [KO:K07375]
103253520  tubulin beta chain-like isoform X1 [KO:K07375]
103272009  tubulin beta-2A chain isoform X3 [KO:K07375]
103272010  tubulin beta-2B chain isoform X2 [KO:K07375]
103252280  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
103272968  MAPK8; mitogen-activated protein kinase 8 isoform X5 [KO:K04440] [EC:2.7.11.24]
103255312  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
103263163  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
103256923  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
103266619  KIF5B; kinesin-1 heavy chain [KO:K10396]
103249209  KLC3; kinesin light chain 3 [KO:K10407]
103249378  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
103268540  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
103256501  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
103273365  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
103257846  caspase-3 [KO:K02187] [EC:3.4.22.56]
103268803  GRM5; metabotropic glutamate receptor 5 [KO:K04604]
103258399  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
103261218  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
103257479  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
103272341  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
103275147  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
103252226  STX1A; syntaxin-1A [KO:K04560]
103261079  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
103256992  inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
103263237  DLG4; disks large homolog 4 isoform X3 [KO:K11828]
103253254  glutamate receptor 1 [KO:K05197]
103274889  GRIA2; glutamate receptor 2 isoform X3 [KO:K05198]
103258957  GRIA3; glutamate receptor 3 [KO:K05199]
103267232  GRIA4; glutamate receptor 4 isoform X2 [KO:K05200]
103258841  TGM2; protein-glutamine gamma-glutamyltransferase 2 isoform X1 [KO:K05625] [EC:2.3.2.13]
103258142  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
103272099  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
103261103  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
103265209  REST; RE1-silencing transcription factor [KO:K09222]
103266070  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
103250763  RCOR1; REST corepressor 1 [KO:K11829]
103269606  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
103269170  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
103275368  histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
103264624  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
103276220  POLR2B; DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
103270504  POLR2C; DNA-directed RNA polymerase II subunit RPB3 isoform X1 [KO:K03011]
103251968  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
103259240  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
103250569  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
103255632  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
103265076  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
103258724  DNA-directed RNA polymerases I, II, and III subunit RPABC3-like isoform X1 [KO:K03016]
103276233  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
110595306  POLR2L; LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
103268112  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
103252117  BDNF; brain-derived neurotrophic factor [KO:K04355]
103271980  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
103273679  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
103267352  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
103268535  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
103269933  TBP; TATA-box-binding protein [KO:K03120]
103251388  TATA box-binding protein-like protein 2 [KO:K03120]
103251434  TATA box-binding protein-like protein 2 [KO:K03120]
103253471  TATA box-binding protein-like protein 2 [KO:K03120]
103253472  TATA box-binding protein-like protein 2 [KO:K03120]
103277012  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
103260226  transcription factor Sp1 [KO:K04684]
103252293  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
103249547  CREB3L3; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
103252136  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
103263755  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
103265465  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
103257302  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
103273492  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X2 [KO:K09047]
103256017  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
103263751  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
103264022  TFAM; transcription factor A, mitochondrial isoform X1 [KO:K11830]
103259542  transcription factor A, mitochondrial-like isoform X2 [KO:K11830]
103250102  NRF1; LOW QUALITY PROTEIN: nuclear respiratory factor 1 [KO:K11831]
103250291  SOD1; superoxide dismutase [Cu-Zn] isoform X2 [KO:K04565] [EC:1.15.1.1]
103276928  SOD2; superoxide dismutase [Mn], mitochondrial isoform X1 [KO:K04564] [EC:1.15.1.1]
103270021  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
103250724  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
103250975  GPX1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
103258981  glutathione peroxidase 2-like [KO:K00432] [EC:1.11.1.9]
103266134  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
103266135  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
103273003  RAB15; ras-related protein Rab-15 isoform X1 [KO:K00432] [EC:1.11.1.9]
103268230  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
103257179  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
103264637  TP53; cellular tumor antigen p53 [KO:K04451]
103256231  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
7944474  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
7944462  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
7944463  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
7944470  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
7944467  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
7944472  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
7944471  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
103251659  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
103258337  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
103272691  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
110596544  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
103276211  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
103265401  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
103277104  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X3 [KO:K03946]
103276463  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947]
103249583  NDUFA4; cytochrome c oxidase subunit NDUFA4 isoform X1 [KO:K03948]
103256865  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
103250410  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
103262643  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X2 [KO:K03949]
103253105  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
103259404  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
103276353  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
103264348  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
103266338  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
103259129  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
103275551  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
103271430  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
103257860  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
103266003  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
103256260  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
103262897  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
110594486  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
103271180  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
103273362  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
103256427  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
103262863  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
103263506  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
103261895  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
103261731  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
103250117  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
103252786  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
103262943  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
103260252  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
103253931  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
103258126  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
103266780  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
103267568  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
103252583  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
103250207  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
103271841  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial-like [KO:K03941] [EC:7.1.1.2]
103252346  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
103271254  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
103275417  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
103257385  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
103251186  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
103260260  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
103268018  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
103254098  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
103265462  cytochrome b-c1 complex subunit Rieske, mitochondrial isoform X1 [KO:K00411] [EC:7.1.1.8]
7944473  CYTB; cytochrome b [KO:K00412]
103272505  cytochrome c1, heme protein, mitochondrial [KO:K00413]
103270632  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
103260331  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
103249224  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
103258555  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
103267371  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 7-like [KO:K00417]
103259262  cytochrome b-c1 complex subunit 8 [KO:K00418]
103271686  cytochrome b-c1 complex subunit 9 [KO:K00419]
103251009  cytochrome b-c1 complex subunit 10 [KO:K00420]
7944464  COX3; cytochrome c oxidase subunit III [KO:K02262]
7944468  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
7944469  COX2; cytochrome c oxidase subunit II [KO:K02261]
103252018  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
103266174  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
103270898  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
103266568  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
103251688  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
103251157  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
110595358  cytochrome c oxidase subunit 6B1-like [KO:K02267]
103263084  cytochrome c oxidase subunit 6B1-like [KO:K02267]
103273566  COX6B; cytochrome c oxidase subunit 6B1-like [KO:K02267]
110596473  cytochrome c oxidase subunit 6B1-like [KO:K02267]
110596696  cytochrome c oxidase subunit 6B1-like [KO:K02267]
110594497  cytochrome c oxidase subunit 6B1-like [KO:K02267]
110595091  cytochrome c oxidase subunit 6C-like [KO:K02268]
110596061  cytochrome c oxidase subunit 6C-like [KO:K02268]
110596332  cytochrome c oxidase subunit 6C-like [KO:K02268]
110596375  cytochrome c oxidase subunit 6C-like [KO:K02268]
110596503  cytochrome c oxidase subunit 6C-like [KO:K02268]
110596727  cytochrome c oxidase subunit 6C-like [KO:K02268]
103268110  cytochrome c oxidase subunit 6C-like [KO:K02268]
103273442  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
103276236  COX7AH; cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
103276500  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
103274382  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
103267420  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
110594887  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
103267388  ATP5A1; ATP synthase subunit alpha, mitochondrial isoform X1 [KO:K02132]
103251114  ATP synthase subunit beta, mitochondrial-like isoform X1 [KO:K02133] [EC:7.1.2.2]
103275145  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
103276345  ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
103270009  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
110594963  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
110596205  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
110596280  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
110594496  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
7944466  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
103271456  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
103251673  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
103253408  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128]
103264736  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
103275931  ATP5H; ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138]
103275990  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
103276196  ATP synthase subunit O, mitochondrial-like [KO:K02137]
103267552  ATP5J; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
7944465  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
103268316  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X2 [KO:K05862]
103263904  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X2 [KO:K15040]
103257263  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
103252175  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
103268812  ADP/ATP translocase 4 [KO:K05863]
103276203  SLC25A5; LOW QUALITY PROTEIN: ADP/ATP translocase 2 [KO:K05863]
103254788  peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
103258177  LOW QUALITY PROTEIN: peptidyl-prolyl cis-trans isomerase F, mitochondrial-like [KO:K09565] [EC:5.2.1.8]
103255326  cytochrome c 2 [KO:K08738]
110596190  cytochrome c, somatic-like [KO:K08738]
103274909  cytochrome c, somatic [KO:K08738]
103251017  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
103260483  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
103273995  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
103274973  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
103262238  proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
103256450  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
103259767  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
103268529  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
103272280  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
103263777  LOW QUALITY PROTEIN: proteasome subunit alpha type-5-like [KO:K02729] [EC:3.4.25.1]
103270408  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
103259028  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
103276553  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
103257362  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
103268852  PSMB3; proteasome subunit beta type-3 isoform X1 [KO:K02735] [EC:3.4.25.1]
103271700  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
103270262  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
103269932  PSMB1; proteasome subunit beta type-1 isoform X1 [KO:K02732] [EC:3.4.25.1]
103274341  PSMB4; proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
103250392  26S protease regulatory subunit 7 [KO:K03061]
103265114  26S protease regulatory subunit 7-like [KO:K03061]
103252809  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
103257018  PSMC4; 26S protease regulatory subunit 6B isoform X1 [KO:K03063]
103275752  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
103256297  PSMC3; 26S protease regulatory subunit 6A [KO:K03065]
103252067  PSMC5; 26S protease regulatory subunit 8 isoform X2 [KO:K03066]
103265072  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
103257341  26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
103267584  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 isoform X1 [KO:K03033]
103262130  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
103271133  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
103251065  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
103254114  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
103272535  26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
103254938  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
103274334  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
103251331  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
103276877  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
103259777  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
110595742  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
103263969  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
103262804  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103252426  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
103256327  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
103251143  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
103272291  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
103265752  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
103264896  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
103268439  ATG101; autophagy-related protein 101 [KO:K19730]
103267779  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
103268777  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
103267783  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 [KO:K17985]
103270696  BECN1; beclin-1 isoform X1 [KO:K08334]
103266086  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
103254413  ATG14; LOW QUALITY PROTEIN: beclin 1-associated autophagy-related key regulator [KO:K17889]
103258390  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
103275750  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
103250165  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
103251110  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
103250649  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
103260005  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
Compound
C00025  L-Glutamate
C00076  Calcium cation
C00238  Potassium cation
C01245  D-myo-Inositol 1,4,5-trisphosphate
C01330  Sodium cation
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
Reference
PMID:15459747
  Authors
Landles C, Bates GP
  Title
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series.
  Journal
EMBO Rep 5:958-63 (2004)
DOI:10.1038/sj.embor.7400250
Reference
PMID:17041811
  Authors
Borrell-Pages M, Zala D, Humbert S, Saudou F
  Title
Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.
  Journal
Cell Mol Life Sci 63:2642-60 (2006)
DOI:10.1007/s00018-006-6242-0
Reference
PMID:18951640
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
DOI:10.1016/j.tins.2008.09.004
Reference
PMID:16288298
  Authors
Cattaneo E, Zuccato C, Tartari M
  Title
Normal huntingtin function: an alternative approach to Huntington's disease.
  Journal
Nat Rev Neurosci 6:919-30 (2005)
DOI:10.1038/nrn1806
Reference
PMID:15336977
  Authors
Bezprozvanny I, Hayden MR
  Title
Deranged neuronal calcium signaling and Huntington disease.
  Journal
Biochem Biophys Res Commun 322:1310-7 (2004)
DOI:10.1016/j.bbrc.2004.08.035
Reference
PMID:14526190
  Authors
Sawa A, Tomoda T, Bae BI
  Title
Mechanisms of neuronal cell death in Huntington's disease.
  Journal
Cytogenet Genome Res 100:287-95 (2003)
DOI:10.1159/000072864
Reference
PMID:12372277
  Authors
Ross CA.
  Title
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
  Journal
Neuron 35:819-22 (2002)
DOI:10.1016/S0896-6273(02)00872-3
Reference
PMID:15242639
  Authors
Ross CA
  Title
Huntington's disease: new paths to pathogenesis.
  Journal
Cell 118:4-7 (2004)
DOI:10.1016/j.cell.2004.06.022
Reference
PMID:24739785
  Authors
Coffey ET
  Title
Nuclear and cytosolic JNK signalling in neurons.
  Journal
Nat Rev Neurosci 15:285-99 (2014)
DOI:10.1038/nrn3729
Reference
PMID:19525941
  Authors
Morfini GA, You YM, Pollema SL, Kaminska A, Liu K, Yoshioka K, Bjorkblom B, Coffey ET, Bagnato C, Han D, Huang CF, Banker G, Pigino G, Brady ST
  Title
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin.
  Journal
Nat Neurosci 12:864-71 (2009)
DOI:10.1038/nn.2346
Reference
PMID:26920688
  Authors
Kumar P, Kumar D, Jha SK, Jha NK, Ambasta RK
  Title
Ion Channels in Neurological Disorders.
  Journal
Adv Protein Chem Struct Biol 103:97-136 (2016)
DOI:10.1016/bs.apcsb.2015.10.006
Reference
PMID:25191533
  Authors
Proft J, Weiss N
  Title
Rectifying rectifier channels in Huntington disease.
  Journal
Commun Integr Biol 7:e29410 (2014)
DOI:10.4161/cib.29410
Reference
PMID:15695335
  Authors
Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I
  Title
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
  Journal
Proc Natl Acad Sci U S A 102:2602-7 (2005)
DOI:10.1073/pnas.0409402102
Reference
PMID:29427371
  Authors
Mackay JP, Nassrallah WB, Raymond LA
  Title
Cause or compensation?-Altered neuronal Ca(2+) handling in Huntington's disease.
  Journal
CNS Neurosci Ther 24:301-310 (2018)
DOI:10.1111/cns.12817
Reference
PMID:11807533
  Authors
Mattson MP.
  Title
Accomplices to neuronal death.
  Journal
Nature 415:377-9 (2002)
DOI:10.1038/415377a
Reference
PMID:17081970
  Authors
McGill JK, Beal MF
  Title
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Journal
Cell 127:465-8 (2006)
DOI:10.1016/j.cell.2006.10.023
Reference
PMID:30941017
  Authors
Intihar TA, Martinez EA, Gomez-Pastor R
  Title
Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1alpha Transcription Factors.
  Journal
Front Cell Neurosci 13:103 (2019)
DOI:10.3389/fncel.2019.00103
Reference
PMID:15996541
  Authors
La Spada AR, Morrison RS
  Title
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
  Journal
Neuron 47:1-3 (2005)
DOI:10.1016/j.neuron.2005.06.023
Reference
PMID:11738471
  Authors
Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
  Journal
Neurochem Int 40:37-52 (2002)
DOI:10.1016/S0197-0186(01)00059-6
Reference
PMID:15996546
  Authors
Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A
  Title
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
  Journal
Neuron 47:29-41 (2005)
DOI:10.1016/j.neuron.2005.06.005
Reference
PMID:11839795
  Authors
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ
  Title
Interaction of Huntington disease protein with transcriptional activator Sp1.
  Journal
Mol Cell Biol 22:1277-87 (2002)
DOI:10.1128/MCB.22.5.1277-1287.2002
Reference
PMID:25270767
  Authors
Vidal RL, Matus S, Bargsted L, Hetz C
  Title
Targeting autophagy in neurodegenerative diseases.
  Journal
Trends Pharmacol Sci 35:583-91 (2014)
DOI:10.1016/j.tips.2014.09.002
Reference
PMID:16522639
  Authors
Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J
  Title
Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1.
  Journal
J Biol Chem 281:14474-85 (2006)
DOI:10.1074/jbc.M600364200
Related
pathway
csyr00190  Oxidative phosphorylation
csyr03020  RNA polymerase
csyr03022  Basal transcription factors
csyr03050  Proteasome
csyr04020  Calcium signaling pathway
csyr04115  p53 signaling pathway
csyr04140  Autophagy - animal
csyr04141  Protein processing in endoplasmic reticulum
csyr04144  Endocytosis
csyr04210  Apoptosis
csyr04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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