KEGG   PATHWAY: etf05016
Entry
etf05016                    Pathway                                
Name
Huntington disease - Echinops telfairi (small Madagascar hedgehog)
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
etf05016  Huntington disease
etf05016

Organism
Echinops telfairi (small Madagascar hedgehog) [GN:etf]
Gene
101663333  CLTA; clathrin light chain A isoform X1 [KO:K04644]
101641351  CLTB; clathrin light chain B isoform X1 [KO:K04645]
101638747  CLTCL1; clathrin heavy chain 2 [KO:K04646]
101656697  CLTC; clathrin heavy chain 1 isoform X4 [KO:K04646]
101648260  HIP1; huntingtin-interacting protein 1 [KO:K04559]
101653065  AP2A1; AP-2 complex subunit alpha-1 isoform X2 [KO:K11824]
101641084  AP2A2; AP-2 complex subunit alpha-2 [KO:K11824]
101643724  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
101659153  AP2M1; AP-2 complex subunit mu [KO:K11826]
101658378  AP2S1; AP-2 complex subunit sigma [KO:K11827]
101639805  HTT; huntingtin [KO:K04533]
101641030  DCTN1; dynactin subunit 1 isoform X3 [KO:K04648]
101651138  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
101648190  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
101639785  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
101658156  DCTN5; dynactin subunit 5 [KO:K10427]
101653255  dynactin subunit 6-like [KO:K10428]
101643224  DCTN6; dynactin subunit 6 [KO:K10428]
101661501  ACTR1B; beta-centractin [KO:K16575]
101653436  ACTR1A; alpha-centractin [KO:K16575]
101659479  ACTR10; LOW QUALITY PROTEIN: actin-related protein 10 [KO:K16576]
105979031  HAP1; huntingtin-associated protein 1 [KO:K04647]
101651076  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
101640394  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
101643208  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
101645365  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
101658242  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
101647835  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
101657359  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
101661940  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
101664376  DNAH2; dynein axonemal heavy chain 2 [KO:K10408]
101650307  DNAH14; dynein axonemal heavy chain 14 [KO:K10408]
101647760  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
101653916  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
101642658  DNAH11; LOW QUALITY PROTEIN: dynein axonemal heavy chain 11 [KO:K10408]
101646258  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
101641029  DNAI1; LOW QUALITY PROTEIN: dynein axonemal intermediate chain 1 [KO:K10409]
101642263  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
101661988  DNAL1; dynein axonemal light chain 1 [KO:K10411]
123521767  DNAL4; dynein axonemal light chain 4 [KO:K10412]
101663498  TUBA4A; tubulin alpha-4A chain [KO:K07374]
101640153  tubulin alpha-1D chain [KO:K07374]
101654209  TUBA8; tubulin alpha-8 chain [KO:K07374]
101660474  tubulin alpha-4A chain [KO:K07374]
101651696  LOW QUALITY PROTEIN: tubulin alpha-1C chain [KO:K07374]
101662276  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
101642837  tubulin alpha-3 chain [KO:K07374]
101654715  tubulin alpha-1C chain-like [KO:K07374]
101664407  tubulin alpha-8 chain [KO:K07374]
101641475  TUBB4A; LOW QUALITY PROTEIN: tubulin beta-4A chain [KO:K07375]
101658886  TUBB6; tubulin beta-6 chain [KO:K07375]
101660038  tubulin beta-4B chain [KO:K07375]
101654809  TUBB3; tubulin beta-3 chain [KO:K07375]
101641139  TUBB2A; tubulin beta-2A chain [KO:K07375]
101649529  TUBB; tubulin beta chain [KO:K07375]
101651329  TUBB1; tubulin beta-1 chain [KO:K07375]
101654493  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
101653598  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
101645489  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
101651348  KIF5B; kinesin-1 heavy chain [KO:K10396]
101639531  KIF5C; kinesin heavy chain [KO:K10396]
101650624  KIF5A; kinesin heavy chain isoform X2 [KO:K10396]
101651085  KLC4; kinesin light chain 4 [KO:K10407]
101660584  KLC3; kinesin light chain 3 [KO:K10407]
101646529  KLC1; kinesin light chain 1 isoform X2 [KO:K10407]
101643439  KLC2; kinesin light chain 2 [KO:K10407]
101643505  IFT57; intraflagellar transport protein 57 homolog isoform X2 [KO:K04638]
101638051  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
101643856  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
101659445  GRM5; metabotropic glutamate receptor 5 [KO:K04604]
101637893  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
101651304  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
101650582  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
101647679  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
101656854  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X3 [KO:K05858] [EC:3.1.4.11]
101657517  STX1A; syntaxin-1A [KO:K04560]
101659681  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
101650392  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X2 [KO:K05208]
101644422  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101654141  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
101645449  DLG4; disks large homolog 4 isoform X7 [KO:K11828]
101643115  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
101648272  GRIA2; glutamate receptor 2 isoform X7 [KO:K05198]
101661759  GRIA3; glutamate receptor 3 [KO:K05199]
101652216  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
101646314  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
101645577  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
101655885  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
101647701  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
101647109  REST; RE1-silencing transcription factor isoform X1 [KO:K09222]
101639140  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
101659171  RCOR1; REST corepressor 1 [KO:K11829]
101664320  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
101664236  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
101652121  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
101646584  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
101649717  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
101653026  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
101651139  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
101664522  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
101640312  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
101654857  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
101663046  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X3 [KO:K03017]
115869693  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 isoform X5 [KO:K03007]
101655131  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
115871031  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
115873548  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
115873754  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
101643198  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
101641939  CREB-binding protein [KO:K04498] [EC:2.3.1.48]
101647070  EP300; histone acetyltransferase p300 isoform X2 [KO:K04498] [EC:2.3.1.48]
101651875  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
101648535  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
101649806  TATA-box-binding protein [KO:K03120]
101655218  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
101650612  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
101656971  SP1; transcription factor Sp1 [KO:K04684]
101646557  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
101653565  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
101648900  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
101654666  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X2 [KO:K09048]
101654039  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X3 [KO:K09048]
101639977  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
101648034  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X2 [KO:K09047]
101653353  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
101656886  PPARG; peroxisome proliferator-activated receptor gamma isoform X3 [KO:K08530]
101647926  TFAM; transcription factor A, mitochondrial [KO:K11830]
101638790  NRF1; nuclear respiratory factor 1 isoform X3 [KO:K11831]
115871255  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
101648393  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
101657497  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
101658621  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
101640617  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
101648659  GPX8; probable glutathione peroxidase 8 [KO:K00432] [EC:1.11.1.9]
101661070  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
101659394  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
101647171  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
101650785  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
101647784  LOW QUALITY PROTEIN: cellular tumor antigen p53-like [KO:K04451]
101656530  TP53; cellular tumor antigen p53 [KO:K04451]
101646711  BAX; apoptosis regulator BAX [KO:K02159]
101659338  BBC3; bcl-2-binding component 3 [KO:K10132]
802330  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
802331  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
802332  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
802333  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
802334  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
802335  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
802336  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
101654446  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
101648307  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
101649115  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X2 [KO:K03944]
115871646  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
101652658  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105979080  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
101642634  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
115871468  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
115871615  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
115871629  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
101654708  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
115873240  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
101654892  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
101660982  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101639113  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
101655451  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101653317  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
101653949  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
101641344  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
101657923  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
101654342  acyl carrier protein, mitochondrial-like [KO:K03955]
101642903  acyl carrier protein, mitochondrial [KO:K03955]
101658197  acyl carrier protein, mitochondrial-like [KO:K03955]
101658798  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
101644308  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
115869492  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12-like [KO:K11352]
101660948  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12-like [KO:K11352]
101657908  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
101644575  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
115868737  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
115873199  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
115869395  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
101656638  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
115872092  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
115872006  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
101654519  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
101643828  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
101640401  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
101643885  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
101647046  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7-like [KO:K03963]
101664420  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
101654859  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
101658030  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
101649968  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
101644338  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
101658539  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
101651179  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
101651054  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
101643499  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
101660829  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
101640767  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
101655488  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
101657196  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101649217  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101647009  NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
101640009  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
101653242  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101660670  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
101660430  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
101640820  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
101644773  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
802338  CYTB; cytochrome b [KO:K00412]
101641779  cytochrome c1, heme protein, mitochondrial [KO:K00413]
101639006  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
101658800  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
101641378  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
101660663  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101655377  cytochrome b-c1 complex subunit 7 [KO:K00417]
115870168  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101655167  cytochrome b-c1 complex subunit 8 [KO:K00418]
101649136  cytochrome b-c1 complex subunit 9 [KO:K00419]
101647848  cytochrome b-c1 complex subunit 10-like [KO:K00420]
101646763  cytochrome b-c1 complex subunit 10 [KO:K00420]
101641433  cytochrome b-c1 complex subunit 10-like [KO:K00420]
802329  COX3; cytochrome c oxidase subunit III [KO:K02262]
802327  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
802328  COX2; cytochrome c oxidase subunit II [KO:K02261]
101662244  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
101644516  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
101663125  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
105979674  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
101645126  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
101657136  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
101641935  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
105979995  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
101656692  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
101660222  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
101640324  cytochrome c oxidase subunit 6B1-like [KO:K02267]
101654913  cytochrome c oxidase subunit 6B1-like [KO:K02267]
101649535  cytochrome c oxidase subunit 6B1-like [KO:K02267]
115868703  cytochrome c oxidase subunit 6B1-like [KO:K02267]
101655402  cytochrome c oxidase subunit 6B2 [KO:K02267]
101657672  cytochrome c oxidase subunit 6B1 [KO:K02267]
115869234  cytochrome c oxidase subunit 6B1-like [KO:K02267]
115868698  cytochrome c oxidase subunit 6C isoform X2 [KO:K02268]
115871260  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
101652550  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
115871755  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
101663987  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
101651542  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
115868983  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
101655666  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
101650318  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
115871644  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
115872385  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
123522755  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
115873769  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
115868264  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
115868682  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
115869232  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
115872049  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
115872554  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
115872007  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
101647414  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
101659808  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
101648654  ATP synthase subunit gamma, mitochondrial-like [KO:K02136]
101640525  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
101641389  ATP synthase subunit delta, mitochondrial [KO:K02134]
101650207  ATP synthase subunit delta, mitochondrial-like [KO:K02134]
115871972  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
115868774  LOW QUALITY PROTEIN: ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
802337  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
101649586  ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
101662086  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
101642029  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
101638576  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
105979491  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
101657495  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
105979765  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
101651440  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
101654301  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
101660847  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
105979066  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
101658560  ATP synthase subunit d, mitochondrial-like [KO:K02138]
101663222  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
101648762  ATP synthase subunit d, mitochondrial-like [KO:K02138]
101643680  ATP synthase subunit d, mitochondrial-like isoform X1 [KO:K02138]
101640972  ATP synthase subunit O, mitochondrial-like [KO:K02137]
101661561  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
101653269  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
101655801  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
101638485  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
802326  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
101656727  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
101653907  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
101649902  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
101642569  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
101638395  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
101643848  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
101648707  ADP/ATP translocase 2 [KO:K05863]
101655091  ADP/ATP translocase 3 [KO:K05863]
101646088  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
101653289  LOW QUALITY PROTEIN: ADP/ATP translocase 2-like [KO:K05863]
101649341  ADP/ATP translocase 2-like [KO:K05863]
101642037  LOW QUALITY PROTEIN: peptidyl-prolyl cis-trans isomerase F, mitochondrial-like [KO:K09565] [EC:5.2.1.8]
101638230  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
101661288  cytochrome c, testis-specific [KO:K08738]
101650600  cytochrome c-like [KO:K08738]
101656765  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
101659735  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
101655936  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
101659004  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
101654154  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
101651352  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
101647600  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
101644957  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
101652319  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
101659790  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
101662625  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
101659272  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101660884  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
101652514  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
101658302  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
101650120  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
101649257  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
101653452  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
101654357  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
101642584  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
101651118  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
101649508  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
101658898  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
101656842  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
101655850  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
101649413  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
101642788  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
101663315  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
101638919  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
101640356  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
101654289  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
101662737  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
101663895  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
101651663  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
101638659  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 14-like [KO:K03030]
101647694  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
101661784  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
105978618  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
101644379  MAP3K10; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
101660158  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101643556  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
101645193  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
101663385  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
101664135  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
101648588  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
101654162  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
101659547  ATG101; autophagy-related protein 101 [KO:K19730]
101643199  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
101656640  autophagy-related protein 13-like [KO:K08331]
101658931  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
101642059  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X3 [KO:K17985]
101638290  BECN1; beclin-1 [KO:K08334]
101643579  beclin-2-like [KO:K08334]
101647419  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
101657057  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
101652595  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101644561  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
101655387  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
101643770  ATG2B; autophagy-related protein 2 homolog B isoform X2 [KO:K17906]
101647959  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
101640179  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
etf00190  Oxidative phosphorylation
etf03020  RNA polymerase
etf03022  Basal transcription factors
etf03050  Proteasome
etf04020  Calcium signaling pathway
etf04115  p53 signaling pathway
etf04140  Autophagy - animal
etf04141  Protein processing in endoplasmic reticulum
etf04144  Endocytosis
etf04210  Apoptosis
etf04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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