KEGG   PATHWAY: nvs05016
Entry
nvs05016                    Pathway                                
Name
Huntington disease - Neogale vison (American mink)
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
nvs05016  Huntington disease
nvs05016

Organism
Neogale vison (American mink) [GN:nvs]
Gene
122917465  CLTA; clathrin light chain A isoform X1 [KO:K04644]
122892504  CLTB; clathrin light chain B isoform X1 [KO:K04645]
122906513  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
122903170  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
122895931  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
122911807  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
122913237  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
122906465  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
122908753  AP2M1; AP-2 complex subunit mu [KO:K11826]
122911910  AP2S1; AP-2 complex subunit sigma [KO:K11827]
122919092  HTT; huntingtin isoform X1 [KO:K04533]
122915002  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
122892421  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
122901764  LOW QUALITY PROTEIN: dynactin subunit 2-like [KO:K10424]
122917511  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
122893945  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
122895652  DCTN5; dynactin subunit 5 [KO:K10427]
122889959  DCTN6; dynactin subunit 6 [KO:K10428]
122901058  ACTR1A; alpha-centractin [KO:K16575]
122916443  ACTR1B; beta-centractin isoform X1 [KO:K16575]
122893393  ACTR10; actin-related protein 10 [KO:K16576]
122907318  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
122904891  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
122905932  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
122906720  DNAH2; dynein axonemal heavy chain 2 [KO:K10408]
122906769  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
122907602  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
122915040  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
122902141  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
122909320  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
122909361  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
122895474  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
122903609  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
122895924  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
122899766  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
122916913  DNAI1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
122907550  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
122893188  DNAL1; dynein axonemal light chain 1 [KO:K10411]
122891101  DNAL4; dynein axonemal light chain 4 [KO:K10412]
122918800  tubulin alpha-8 chain [KO:K07374]
122897974  tubulin alpha-4A chain [KO:K07374]
122891693  TUBAL3; LOW QUALITY PROTEIN: tubulin alpha chain-like 3 [KO:K07374]
122892569  TUBA1A; tubulin alpha-1A chain [KO:K07374]
122892570  tubulin alpha-1C chain [KO:K07374]
122892581  TUBA1B; tubulin alpha-1B chain [KO:K07374]
122892638  TUBA8; tubulin alpha-8 chain [KO:K07374]
122902333  tubulin alpha-4A chain [KO:K07374]
122903183  tubulin alpha-3 chain isoform X1 [KO:K07374]
122903185  tubulin alpha-3 chain isoform X1 [KO:K07374]
122912054  TUBB; tubulin beta chain [KO:K07375]
122912366  TUBB2A; tubulin beta-2A chain isoform X1 [KO:K07375]
122914643  tubulin beta-3 chain [KO:K07375]
122915528  TUBB1; tubulin beta-1 chain [KO:K07375]
122909818  TUBB4A; tubulin beta-4A chain [KO:K07375]
122903131  TUBB6; tubulin beta-6 chain [KO:K07375]
122917058  TUBB4B; tubulin beta-4B chain [KO:K07375]
122889452  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
122900866  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
122915651  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
122891838  KIF5A; kinesin heavy chain [KO:K10396]
122892362  KIF5B; kinesin-1 heavy chain [KO:K10396]
122902669  KIF5C; kinesin heavy chain [KO:K10396]
122911946  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
122913143  KLC2; kinesin light chain 2 [KO:K10407]
122908415  KLC4; kinesin light chain 4 [KO:K10407]
122893561  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
122908483  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
122902190  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
122889990  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
122912504  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
122917675  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
122913059  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
122916255  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
122916256  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
122895707  STX1A; syntaxin-1A [KO:K04560]
122917048  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
122917067  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
122891466  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
122909251  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
122906683  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
122893731  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
122889500  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
122896322  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
122912242  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
122916085  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
122895302  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
122912839  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
122918342  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
122890154  REST; RE1-silencing transcription factor [KO:K09222]
122897967  paired amphipathic helix protein Sin3a-like isoform X1 [KO:K11644]
122898043  SIN3A; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
122893588  RCOR1; REST corepressor 1 [KO:K11829]
122912289  histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
122899699  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
122903407  HDAC2; histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
122906709  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
122890544  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
122902606  LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB3-like [KO:K03011]
122911413  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
122902770  POLR2D; DNA-directed RNA polymerase II subunit RPB4 isoform X1 [KO:K03012]
122909964  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
122891283  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
122913018  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
122908764  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
122913830  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
122898836  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
122913236  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
122895535  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
122905541  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
122912801  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
122891583  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
122895878  CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
122915848  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
122902967  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
122896830  TATA box-binding protein-like 1 [KO:K03120]
122897956  TATA-box-binding protein [KO:K03120]
122906497  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
122893317  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
122902454  TATA-box-binding protein [KO:K03120]
122892412  SP1; transcription factor Sp1 [KO:K04684]
122902244  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
122905111  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
122912878  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
122910819  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
122917481  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
122918221  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
122904940  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
122890143  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
122909195  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
122900688  TFAM; transcription factor A, mitochondrial [KO:K11830]
122905062  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
122908345  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
122902858  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
122889545  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
122899977  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
122914748  epididymal secretory glutathione peroxidase-like [KO:K00432] [EC:1.11.1.9]
122914764  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
122893897  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
122893953  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
122910663  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
122919167  UCP1; mitochondrial brown fat uncoupling protein 1 isoform X1 [KO:K08769]
122906719  TP53; cellular tumor antigen p53 [KO:K04451]
122911843  BAX; apoptosis regulator BAX [KO:K02159]
122911902  BBC3; bcl-2-binding component 3 [KO:K10132]
14841852  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
14841840  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
14841846  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
14841848  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
14841847  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
14841849  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
14841850  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
122913194  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
122903032  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
122908267  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
122897149  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
122915592  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
122890557  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
122911678  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
122904860  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
122897372  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
122891482  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
122894691  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
122903641  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
122905031  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
122891864  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
122909803  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
122917290  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
122891108  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
122902465  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
122895608  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
122909833  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
122891747  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
122909517  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
122894479  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
122905137  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
122902186  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
122908543  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
122909800  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
122908719  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
122917534  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
122909545  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
122901022  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
122904311  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
122896181  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
122897625  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
122902230  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
122918373  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
122912907  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
122894329  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
122897039  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
122906214  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
122899787  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
122908823  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
122896151  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
122909951  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
122913199  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
122919183  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
122892467  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
122903016  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
122912547  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
122896387  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
122899501  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
122918379  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
122910054  LOW QUALITY PROTEIN: succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
122912281  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
122910115  cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
122911488  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
14841851  CYTB; cytochrome b [KO:K00412]
122904230  cytochrome c1, heme protein, mitochondrial [KO:K00413]
122909454  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
122895793  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
122899923  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
122889565  cytochrome b-c1 complex subunit 7-like [KO:K00417]
122899957  cytochrome b-c1 complex subunit 7-like [KO:K00417]
122892438  cytochrome b-c1 complex subunit 7-like [KO:K00417]
122904422  cytochrome b-c1 complex subunit 7 [KO:K00417]
122889942  cytochrome b-c1 complex subunit 8-like [KO:K00418]
122891839  cytochrome b-c1 complex subunit 8 [KO:K00418]
122902031  cytochrome b-c1 complex subunit 9 [KO:K00419]
122909942  cytochrome b-c1 complex subunit 10 [KO:K00420]
14841845  COX3; cytochrome c oxidase subunit III [KO:K02262]
14841841  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
14841842  COX2; cytochrome c oxidase subunit II [KO:K02261]
122915598  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
122911162  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
122893690  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
122915687  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
122894239  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
122895433  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
122903357  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
122912869  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6B1-like [KO:K02267]
122891980  cytochrome c oxidase subunit 6B1-like [KO:K02267]
122913938  cytochrome c oxidase subunit 6B2 [KO:K02267]
122911559  cytochrome c oxidase subunit 6B1 [KO:K02267]
122896390  cytochrome c oxidase subunit 6C [KO:K02268]
122914073  cytochrome c oxidase subunit 6C-like [KO:K02268]
122905394  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
122897493  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
122915125  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
122903450  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
122911585  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
122897159  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
122889784  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
122900125  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
122897479  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
122910059  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
122917769  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
122914556  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
122902906  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
122891405  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
122890845  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
122909967  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
122906212  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
122901826  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
122915928  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
122904096  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
14841844  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
122900323  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
122896727  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
122896778  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
122906610  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
122891642  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
122908798  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
122902615  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
122906004  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
122900900  ATP synthase subunit d, mitochondrial-like [KO:K02138]
122908319  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
122896797  ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
122896839  ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
122908364  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
14841843  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
122904870  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
122891745  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
122900317  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
122900818  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
122889854  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
122889978  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
122897566  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
122890114  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
122916940  LOW QUALITY PROTEIN: ADP/ATP translocase 2-like [KO:K05863]
122896262  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
122900832  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
122904917  cytochrome c [KO:K08738]
122902631  cytochrome c, testis-specific [KO:K08738]
122902991  cytochrome c-like [KO:K08738]
122892476  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
122899479  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
122893169  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
122904639  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
122893455  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
122901943  PSMA8; LOW QUALITY PROTEIN: proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
122915916  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
122900289  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
122905440  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
122912744  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
122893781  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
122907750  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
122917262  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
122906316  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
122899744  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
122893551  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
122902455  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
122900507  PSMB4; proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
122904734  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
122898887  26S proteasome regulatory subunit 4-like [KO:K03062]
122893994  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
122912030  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
122898006  26S proteasome regulatory subunit 6B [KO:K03063]
122914576  26S proteasome regulatory subunit 10B-like [KO:K03064]
122894431  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
122912902  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
122906087  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
122908760  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
122902404  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
122906292  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
122903045  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
122906065  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
122906450  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
122909293  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
122911287  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
122913204  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
122900500  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
122902532  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
122911633  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
122915911  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
122904841  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
122912031  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
122906080  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
122917083  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
122906270  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
122909795  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
122899446  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
122903104  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
122906817  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
122907396  ULK2; LOW QUALITY PROTEIN: serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
122891893  ATG101; autophagy-related protein 101 [KO:K19730]
122912884  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
122904603  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
122912882  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
122906173  BECN1; beclin-1 isoform X1 [KO:K08334]
122917964  BECN2; beclin-2 [KO:K08334]
122908951  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
122892975  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
122900714  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
122902914  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
122913078  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
122894203  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
122906054  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
122895255  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
nvs00190  Oxidative phosphorylation
nvs03020  RNA polymerase
nvs03022  Basal transcription factors
nvs03050  Proteasome
nvs04020  Calcium signaling pathway
nvs04115  p53 signaling pathway
nvs04140  Autophagy - animal
nvs04141  Protein processing in endoplasmic reticulum
nvs04144  Endocytosis
nvs04210  Apoptosis
nvs04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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