KEGG   PATHWAY: mthb05016
Entry
mthb05016                   Pathway                                
Name
Huntington disease - Macaca thibetana thibetana (Pere David's macaque)
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
mthb05016  Huntington disease
mthb05016

Organism
Macaca thibetana thibetana (Pere David's macaque) [GN:mthb]
Gene
126938091  clathrin light chain A isoform X1 [KO:K04644]
126956365  clathrin light chain B isoform X1 [KO:K04645]
126939365  clathrin heavy chain 1 isoform X1 [KO:K04646]
126929591  clathrin heavy chain 2 isoform X1 [KO:K04646]
126950327  huntingtin-interacting protein 1 isoform X1 [KO:K04559]
126943093  AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
126935825  AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
126939516  AP-2 complex subunit beta isoform X1 [KO:K11825]
126948412  AP-2 complex subunit mu [KO:K11826]
126943511  AP-2 complex subunit sigma [KO:K11827]
126955552  huntingtin isoform X1 [KO:K04533]
126934009  dynactin subunit 1 isoform X1 [KO:K04648]
126930390  dynactin subunit 2 [KO:K10424]
126937424  dynactin subunit 3 [KO:K10425]
126956603  dynactin subunit 4 isoform X1 [KO:K10426]
126944401  dynactin subunit 5 [KO:K10427]
126961805  dynactin subunit 6 [KO:K10428]
126962755  alpha-centractin [KO:K16575]
126934331  beta-centractin isoform X1 [KO:K16575]
126959921  actin-related protein 10 [KO:K16576]
126938304  LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
126930586  dynein axonemal heavy chain 10 isoform X1 [KO:K10408]
126937018  dynein axonemal heavy chain 14 isoform X1 [KO:K10408]
126943692  dynein axonemal heavy chain 3 isoform X1 [KO:K10408]
126956202  dynein axonemal heavy chain 5 [KO:K10408]
126932964  LOW QUALITY PROTEIN: dynein axonemal heavy chain 7 [KO:K10408]
126939151  dynein axonemal heavy chain 17 [KO:K10408]
126939570  dynein axonemal heavy chain 9 isoform X1 [KO:K10408]
126939635  dynein axonemal heavy chain 2 isoform X1 [KO:K10408]
126950860  dynein axonemal heavy chain 11 [KO:K10408]
126934160  dynein axonemal heavy chain 6 isoform X1 [KO:K10408]
126952706  dynein axonemal heavy chain 8 [KO:K10408]
126948767  dynein axonemal heavy chain 1 [KO:K10408]
126948795  dynein axonemal heavy chain 12 isoform X1 [KO:K10408]
126960746  axonemal dynein light intermediate polypeptide 1 [KO:K10410]
126937387  dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
126939905  dynein axonemal intermediate chain 2 isoform X1 [KO:K11143]
126959569  dynein axonemal light chain 1 isoform X1 [KO:K10411]
126963780  dynein axonemal light chain 4 [KO:K10412]
126962351  tubulin alpha chain-like 3 [KO:K07374]
126930834  tubulin alpha-1B chain [KO:K07374]
126930835  tubulin alpha-1A chain isoform X1 [KO:K07374]
126930836  tubulin alpha-1C chain [KO:K07374]
126932997  tubulin alpha-4A chain isoform X1 [KO:K07374]
126947927  tubulin alpha-3E chain [KO:K07374]
126929646  tubulin alpha-3 chain [KO:K07374]
126929647  tubulin alpha-8 chain [KO:K07374]
126942625  tubulin beta-4A chain [KO:K07375]
126961754  tubulin beta-8 chain [KO:K07375]
126936994  tubulin beta-4B chain [KO:K07375]
126956508  tubulin beta-8 chain-like [KO:K07375]
126941215  tubulin beta-6 chain isoform X1 [KO:K07375]
126947941  tubulin beta-8 chain-like [KO:K07375]
126952529  tubulin beta-2A chain isoform X1 [KO:K07375]
126953079  tubulin beta chain isoform X1 [KO:K07375]
126929351  tubulin beta-1 chain [KO:K07375]
126944112  uncharacterized protein LOC126944112 [KO:K07375]
126954697  mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
126963142  mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
126956917  mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
126930365  kinesin heavy chain [KO:K10396]
126963379  kinesin-1 heavy chain isoform X1 [KO:K10396]
126932906  kinesin heavy chain [KO:K10396]
126943454  kinesin light chain 3 isoform X1 [KO:K10407]
126952395  kinesin light chain 4 isoform X1 [KO:K10407]
126959414  kinesin light chain 1 isoform X1 [KO:K10407]
126935351  kinesin light chain 2 [KO:K10407]
126949270  intraflagellar transport protein 57 homolog [KO:K04638]
126932110  caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
126954341  caspase-3 [KO:K02187] [EC:3.4.22.56]
126934742  metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
126937577  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
126964414  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
126959796  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
126964415  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
126935554  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
126950389  syntaxin-1A isoform X1 [KO:K04560]
126936874  voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
126936897  glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
126930739  glutamate receptor ionotropic, NMDA 2B [KO:K05210]
126947659  inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
126939676  disks large homolog 4 isoform X1 [KO:K11828]
126956721  glutamate receptor 1 isoform X1 [KO:K05197]
126954308  glutamate receptor 2 isoform X1 [KO:K05198]
126946092  glutamate receptor 3 [KO:K05199]
126936243  glutamate receptor 4 isoform X1 [KO:K05200]
126964190  protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
126957082  excitatory amino acid transporter 1 [KO:K05614]
126936217  excitatory amino acid transporter 2 isoform X1 [KO:K05613]
126936020  ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
126955058  RE1-silencing transcription factor isoform X1 [KO:K09222]
126959988  paired amphipathic helix protein Sin3a [KO:K11644]
126960085  REST corepressor 1 [KO:K11829]
126957492  histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
126952258  histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
126939642  DNA-directed RNA polymerase II subunit RPB1 isoform X1 [KO:K03006] [EC:2.7.7.6]
126955057  DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
126943880  DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
126932587  DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
126935672  LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
126963777  DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
126935513  DNA-directed RNA polymerase II subunit RPB7 isoform X1 [KO:K03015]
126948433  DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
126942111  DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
126953354  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
126935878  DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
126950392  DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
126949371  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
126961152  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
126934686  brain-derived neurotrophic factor isoform X1 [KO:K04355]
126963565  histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
126944569  CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
126940972  transcription initiation factor TFIID subunit 4B [KO:K03129]
126929065  transcription initiation factor TFIID subunit 4 [KO:K03129]
126952481  TATA-box-binding protein [KO:K03120]
126959340  TATA box-binding protein-like 2 [KO:K03120]
126952889  TATA box-binding protein-like 1 [KO:K03120]
126931105  transcription factor Sp1 isoform X1 [KO:K04684]
126933094  cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
126942639  cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
126938066  cyclic AMP-responsive element-binding protein 3 [KO:K09048]
126951142  cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
126934237  cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
126935751  cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
126950050  cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
126954797  peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
126949058  peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
126962161  transcription factor A, mitochondrial isoform X1 [KO:K11830]
126949761  nuclear respiratory factor 1 isoform X1 [KO:K11831]
126951704  superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
126953664  superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
126962530  glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
126956614  glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
126957168  probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
126959762  glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
126953142  epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
126953144  glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
126948738  glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
126954613  mitochondrial brown fat uncoupling protein 1 [KO:K08769]
126939707  cellular tumor antigen p53 isoform X1 [KO:K04451]
126943600  apoptosis regulator BAX isoform X1 [KO:K02159]
126943534  bcl-2-binding component 3 [KO:K10132]
126947324  NADH-ubiquinone oxidoreductase chain 5-like [KO:K03883] [EC:7.1.1.2]
126935373  NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
126930135  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial-like [KO:K03943] [EC:7.1.1.2]
126941129  NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
126950655  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
126932207  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
126946139  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
126941467  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
126957346  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
126950258  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
126942379  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
126942872  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
126943346  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947]
126944267  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
126956821  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
126930414  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
126962403  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126956036  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126949986  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
126963966  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126945886  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126958389  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126960684  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
126949375  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
126938104  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
126949807  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
126960621  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
126953759  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
126963639  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
126942854  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951]
126937961  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
126931739  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
126932556  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
126937587  acyl carrier protein, mitochondrial-like [KO:K03955]
126944402  acyl carrier protein, mitochondrial [KO:K03955]
126951198  acyl carrier protein, mitochondrial-like [KO:K03955]
126942837  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
126932729  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956]
126930310  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
126942362  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
126959705  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
126948765  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
126949662  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
126932125  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
126934097  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
126958757  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
126934260  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
126949249  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
126939064  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
126935141  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
126948391  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
126937431  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
126942449  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
126963088  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
126944751  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
126939420  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
126950876  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
126934694  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964]
126961397  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
126944752  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
126930302  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial-like [KO:K11351]
126945660  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
126932178  NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
126935729  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
126935957  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial-like [KO:K03935] [EC:7.1.1.2]
126936392  NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
126945830  NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial-like [KO:K03936] [EC:7.1.1.2]
126956863  NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
126938277  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
126963401  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
126960994  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 isoform X1 [KO:K03938]
126961122  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
126957428  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
126942790  NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
126935402  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
126954622  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
126935701  NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
126956207  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial-like [KO:K00234] [EC:1.3.5.1]
126957442  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
126955714  succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
126961462  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
126936808  succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
126962850  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
126956192  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
126950253  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
126961220  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236]
126935807  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
126930673  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
126943623  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
126960981  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
126948676  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
126944495  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
126954785  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
126961549  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
126937178  cytochrome b-c1 complex subunit 6, mitochondrial-like [KO:K00416]
126934429  cytochrome b-c1 complex subunit 6, mitochondrial-like [KO:K00416]
126960839  cytochrome b-c1 complex subunit 7 [KO:K00417]
126954793  cytochrome b-c1 complex subunit 8-like [KO:K00418]
126956035  cytochrome b-c1 complex subunit 8 [KO:K00418]
126963991  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
126942883  cytochrome b-c1 complex subunit 10 [KO:K00420]
126944333  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
126964308  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
126934981  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
126959142  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
126960028  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
126936073  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
126956094  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
126934339  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
126944266  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
126946802  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
126943264  cytochrome c oxidase subunit 6B2 [KO:K02267]
126939820  cytochrome c oxidase subunit 6B1-like [KO:K02267]
126941943  cytochrome c oxidase subunit 6B1 [KO:K02267]
126954498  cytochrome c oxidase subunit 6C [KO:K02268]
126961151  cytochrome c oxidase subunit 6C [KO:K02268]
126946168  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
126934205  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
126946829  cytochrome c oxidase subunit 7A-related protein, mitochondrial-like [KO:K02270]
126951565  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
126952142  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
126942121  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
126955253  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
126946324  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
126942189  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
126930116  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126943807  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126956722  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126938620  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126957169  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126957397  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
126958011  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126933618  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
126946671  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126958869  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126952688  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126960733  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
126959711  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
126935615  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
126941410  ATP synthase subunit alpha, mitochondrial [KO:K02132]
126930379  ATP synthase subunit beta, mitochondrial isoform X1 [KO:K02133] [EC:7.1.2.2]
126962920  ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
126942994  ATP synthase subunit delta, mitochondrial isoform X1 [KO:K02134]
126929356  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
126930054  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
126955326  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
126951623  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
126954576  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
126942840  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
126930915  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
126931179  ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
126963773  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
126932708  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
126964152  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
126939892  ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
126940287  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
126959053  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
126949063  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
126954618  ATP synthase subunit d, mitochondrial-like [KO:K02138]
126939263  ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138]
126934985  ATP synthase subunit d, mitochondrial-like [KO:K02138]
126933171  ATP synthase subunit O, mitochondrial-like [KO:K02137]
126950727  ATP synthase subunit O, mitochondrial [KO:K02137]
126950759  ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
126956005  voltage-dependent anion-selective channel protein 1 [KO:K05862]
126962446  voltage-dependent anion-selective channel protein 2 [KO:K15040]
126960768  voltage-dependent anion-selective channel protein 3 [KO:K15041]
126955617  ADP/ATP translocase 4 isoform X1 [KO:K05863]
126955724  ADP/ATP translocase 1 [KO:K05863]
126946121  ADP/ATP translocase 2 [KO:K05863]
126946856  ADP/ATP translocase 3 [KO:K05863]
126947143  ADP/ATP translocase 3 [KO:K05863]
126962461  peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
126949081  cytochrome c-like [KO:K08738]
126961774  cytochrome c-like [KO:K08738]
126936610  cytochrome c-like [KO:K08738]
126937973  cytochrome c-like [KO:K08738]
126944405  cytochrome c-like [KO:K08738]
126932751  cytochrome c-like [KO:K08738]
126951393  cytochrome c [KO:K08738]
126947125  cytochrome c-like [KO:K08738]
126941401  cytochrome c-like [KO:K08738]
126952962  cytochrome c-like [KO:K08738]
126942126  cytochrome c-like [KO:K08738]
126931282  apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
126929188  LOW QUALITY PROTEIN: caspase-9 [KO:K04399] [EC:3.4.22.62]
126959174  proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
126950177  proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
126960062  proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
126940977  proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
126929078  proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
126930104  proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
126936605  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
126950077  LOW QUALITY PROTEIN: proteasome subunit alpha type-3-like [KO:K02727] [EC:3.4.25.1]
126959922  proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
126939764  proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
126937216  proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
126938564  proteasome subunit beta type-3 isoform X1 [KO:K02735] [EC:3.4.25.1]
126959288  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
126960876  proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
126958306  proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
126952482  proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
126932440  proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
126949873  26S proteasome regulatory subunit 7 [KO:K03061]
126949685  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
126959662  26S proteasome regulatory subunit 4 [KO:K03062]
126942045  26S proteasome regulatory subunit 6B [KO:K03063]
126958521  26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
126936384  26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
126939552  26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
126948430  26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
126932353  26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
126938459  26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
126930686  26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
126938351  26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
126938990  26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
126948827  26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
126944080  26S proteasome non-ATPase regulatory subunit 7 isoform X1 [KO:K03038]
126936318  26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
126932170  26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
126932948  26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
126942058  26S proteasome non-ATPase regulatory subunit 8 isoform X1 [KO:K03031]
126929075  proteasomal ubiquitin receptor ADRM1 [KO:K06691]
126949968  26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
126941969  mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
126939534  serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
126936973  TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
126952847  mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
126942652  dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
126952739  serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
126930540  serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
126939023  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
126931061  autophagy-related protein 101 [KO:K19730]
126935748  autophagy-related protein 13 isoform X1 [KO:K08331]
126961411  RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
126935738  activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
126942963  beclin-2 [KO:K08334]
126938472  beclin-1 isoform X1 [KO:K08334]
126947014  phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
126959336  beclin 1-associated autophagy-related key regulator [KO:K17889]
126962156  nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
126941247  phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
126959628  autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
126935549  autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
126938352  WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
126950483  WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [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
mthb00190  Oxidative phosphorylation
mthb03020  RNA polymerase
mthb03022  Basal transcription factors
mthb03050  Proteasome
mthb04020  Calcium signaling pathway
mthb04115  p53 signaling pathway
mthb04140  Autophagy - animal
mthb04141  Protein processing in endoplasmic reticulum
mthb04144  Endocytosis
mthb04210  Apoptosis
mthb04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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