KEGG   PATHWAY: elk05016
Entry
elk05016                    Pathway                                
Name
Huntington disease - Enhydra lutris kenyoni (northern sea otter)
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
elk05016  Huntington disease
elk05016

Organism
Enhydra lutris kenyoni (northern sea otter) [GN:elk]
Gene
111145614  clathrin light chain A isoform X1 [KO:K04644]
111140858  clathrin light chain B isoform X1 [KO:K04645]
111142671  clathrin heavy chain 2 [KO:K04646]
111151260  clathrin heavy chain 1 isoform X1 [KO:K04646]
111145730  huntingtin-interacting protein 1 [KO:K04559]
111149627  AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
111160560  AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
111151383  AP-2 complex subunit beta isoform X1 [KO:K11825]
111148498  AP-2 complex subunit mu isoform X1 [KO:K11826]
111160803  AP-2 complex subunit sigma isoform X1 [KO:K11827]
111149245  LOW QUALITY PROTEIN: huntingtin [KO:K04533]
111147910  dynactin subunit 1 isoform X1 [KO:K04648]
111160098  dynactin subunit 2 isoform X1 [KO:K10424]
111145732  dynactin subunit 3 isoform X1 [KO:K10425]
111141168  dynactin subunit 4 isoform X1 [KO:K10426]
111162315  dynactin subunit 5 [KO:K10427]
111144608  dynactin subunit 6 [KO:K10428]
111144862  alpha-centractin [KO:K16575]
111155921  beta-centractin [KO:K16575]
111160254  actin-related protein 10 [KO:K16576]
111151488  LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
111147958  dynein heavy chain 6, axonemal [KO:K10408]
111147239  dynein heavy chain 11, axonemal [KO:K10408]
111150822  dynein heavy chain 14, axonemal [KO:K10408]
111161130  dynein heavy chain 9, axonemal [KO:K10408]
111161163  dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
111143860  dynein heavy chain 12, axonemal [KO:K10408]
111145753  dynein heavy chain 3, axonemal [KO:K10408]
111156345  LOW QUALITY PROTEIN: dynein heavy chain 10, axonemal-like [KO:K10408]
111143889  dynein heavy chain 1, axonemal [KO:K10408]
111160287  dynein heavy chain 17, axonemal [KO:K10408]
111156405  dynein heavy chain 7, axonemal [KO:K10408]
111147554  dynein heavy chain 8, axonemal [KO:K10408]
111153413  dynein heavy chain 5, axonemal [KO:K10408]
111153412  dynein heavy chain 5, axonemal-like [KO:K10408]
111143650  LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
111145979  dynein intermediate chain 1, axonemal [KO:K10409]
111158169  dynein intermediate chain 2, axonemal [KO:K11143]
111148699  dynein light chain 1, axonemal [KO:K10411]
111157606  dynein light chain 4, axonemal isoform X1 [KO:K10412]
111143142  tubulin alpha-4A chain [KO:K07374]
111142067  tubulin alpha-1A chain-like [KO:K07374]
111157712  tubulin alpha-1B chain [KO:K07374]
111157713  tubulin alpha-1A chain [KO:K07374]
111158883  tubulin alpha-1A chain-like [KO:K07374]
111159721  tubulin alpha-8 chain isoform X1 [KO:K07374]
111157791  tubulin alpha-1C chain [KO:K07374]
111144980  putative tubulin-like protein alpha-4B [KO:K07374]
111151071  tubulin alpha-8 chain isoform X1 [KO:K07374]
111158635  LOW QUALITY PROTEIN: tubulin alpha chain-like 3 [KO:K07374]
111142377  tubulin alpha-3 chain [KO:K07374]
111142381  tubulin alpha-3 chain [KO:K07374]
111141694  tubulin beta-1 chain [KO:K07375]
111142513  tubulin beta-6 chain isoform X1 [KO:K07375]
111161751  tubulin beta-4A chain isoform X1 [KO:K07375]
111156662  tubulin beta-2A chain isoform X1 [KO:K07375]
111152799  tubulin beta-3 chain [KO:K07375]
111156744  tubulin beta-2B chain isoform X1 [KO:K07375]
111146420  tubulin beta-4B chain isoform X1 [KO:K07375]
111152509  tubulin beta chain isoform X1 [KO:K07375]
111144860  mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
111158897  mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
111154016  mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
111155551  kinesin-1 heavy chain [KO:K10396]
111160138  kinesin heavy chain isoform X1 [KO:K10396]
111142050  kinesin heavy chain [KO:K10396]
111160692  kinesin light chain 3 isoform X1 [KO:K10407]
111148551  kinesin light chain 1 isoform X1 [KO:K10407]
111149675  kinesin light chain 2 isoform X1 [KO:K10407]
111147688  kinesin light chain 4 isoform X1 [KO:K10407]
111151964  intraflagellar transport protein 57 homolog [KO:K04638]
111141026  caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
111144607  caspase-3 [KO:K02187] [EC:3.4.22.56]
111154498  metabotropic glutamate receptor 5 [KO:K04604]
111153498  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
111141432  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
111162302  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
111141620  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
111150005  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
111145554  syntaxin-1A isoform X1 [KO:K04560]
111145672  voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
111145480  glutamate receptor ionotropic, NMDA 1-like [KO:K05208]
111145775  glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
111142166  glutamate receptor ionotropic, NMDA 2B [KO:K05210]
111144005  inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
111161072  disks large homolog 4 isoform X1 [KO:K11828]
111140968  glutamate receptor 1 isoform X1 [KO:K05197]
111153762  glutamate receptor 2 isoform X1 [KO:K05198]
111157250  glutamate receptor 3 [KO:K05199]
111153760  glutamate receptor 4 isoform X1 [KO:K05200]
111141771  protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
111140916  excitatory amino acid transporter 1 isoform X1 [KO:K05614]
111149732  excitatory amino acid transporter 2 [KO:K05613]
111138946  ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
111149307  RE1-silencing transcription factor [KO:K09222]
111153299  paired amphipathic helix protein Sin3a [KO:K11644]
111148509  REST corepressor 1 [KO:K11829]
111142974  histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
111158262  histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
111161088  LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB1-like [KO:K03006] [EC:2.7.7.6]
111161116  LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB1-like [KO:K03006] [EC:2.7.7.6]
111149241  DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
111157404  DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
111160866  DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
111161893  DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
111157687  DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
111149658  DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
111148606  DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
111161572  DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
111148194  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
111150095  DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
111147490  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
111145948  DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
111145838  DNA-directed RNA polymerase II subunit RPB11-a-like [KO:K03008]
111146668  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
111150176  LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
111157665  histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
111156131  LOW QUALITY PROTEIN: CREB-binding protein [KO:K04498] [EC:2.3.1.48]
111142522  transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
111141505  transcription initiation factor TFIID subunit 4 [KO:K03129]
111147566  TATA box-binding protein-like protein 1 [KO:K03120]
111158703  TATA-box-binding protein [KO:K03120]
111140634  TATA box-binding protein-like protein 2 [KO:K03120]
111157856  transcription factor Sp1 isoform X1 [KO:K04684]
111147575  cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
111151973  cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
111146280  cyclic AMP-responsive element-binding protein 3 [KO:K09048]
111150050  cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
111138943  cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
111161856  cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
111147321  cyclic AMP-responsive element-binding protein 5 [KO:K09047]
111149050  peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
111144031  peroxisome proliferator-activated receptor gamma [KO:K08530]
111145309  transcription factor A, mitochondrial isoform X1 [KO:K11830]
111151940  nuclear respiratory factor 1 isoform X1 [KO:K11831]
111155698  superoxide dismutase [Cu-Zn] isoform X1 [KO:K04565] [EC:1.15.1.1]
111158728  superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
111156930  probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
111158122  glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
111162615  glutathione peroxidase 6-like [KO:K00432] [EC:1.11.1.9]
111143439  glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
111143890  glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
111162616  epididymal secretory glutathione peroxidase isoform X2 [KO:K00432] [EC:1.11.1.9]
111140869  glutathione peroxidase 3 isoform X1 [KO:K00432] [EC:1.11.1.9]
111149149  mitochondrial brown fat uncoupling protein 1 [KO:K08769]
111161133  cellular tumor antigen p53 [KO:K04451]
111160657  apoptosis regulator BAX isoform X1 [KO:K02159]
111160670  LOW QUALITY PROTEIN: bcl-2-binding component 3 [KO:K10132]
111149612  NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
111142406  NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
111155738  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
111157253  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
111140937  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111139570  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111152555  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
111147374  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111160211  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
111154239  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
111151938  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
111155596  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
111157547  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
111161756  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
111145557  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
111159811  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
111161328  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
111162309  acyl carrier protein, mitochondrial [KO:K03955]
111161769  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
111142148  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
111143867  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
111148723  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111151936  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
111141342  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
111148802  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 isoform X1 [KO:K03960]
111147306  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
111146284  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
111162542  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
111145242  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
111146716  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
111156034  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
111150594  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
111141377  NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
111138869  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
111149396  NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
111141116  NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
111142993  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
111153450  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
111161695  NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
111149681  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
111149131  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
111154340  NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
111153400  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
111154638  succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
111138865  succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
111153893  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
111139384  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
111146626  cytochrome c1, heme protein, mitochondrial [KO:K00413]
111143869  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
111162256  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
111142816  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
111146514  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
111141027  cytochrome b-c1 complex subunit 8 isoform X1 [KO:K00418]
111160042  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
111161710  cytochrome b-c1 complex subunit 10 [KO:K00420]
111153043  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
111141707  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
111153215  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
111155858  cytochrome c oxidase subunit 5B, mitochondrial isoform X1 [KO:K02265]
111162190  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
111156401  cytochrome c oxidase subunit 6A1, mitochondrial isoform X1 [KO:K02266]
111161463  cytochrome c oxidase subunit 6B1 isoform X1 [KO:K02267]
111155471  cytochrome c oxidase subunit 6B1-like [KO:K02267]
111139590  cytochrome c oxidase subunit 6B2 [KO:K02267]
111147664  cytochrome c oxidase subunit 6C [KO:K02268]
111146812  cytochrome c oxidase subunit 6C [KO:K02268]
111161502  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
111147649  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
111158437  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
111146524  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111156868  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
111160926  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
111149314  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
111149315  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
111148335  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111152225  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111160948  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
111149630  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
111152453  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
111142536  ATP synthase subunit alpha, mitochondrial isoform X1 [KO:K02132]
111160107  ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
111158608  ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
111161765  ATP synthase subunit delta, mitochondrial [KO:K02134]
111152423  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
111141583  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
111143342  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
111150035  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
111157857  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
111151344  ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
111160337  ATP synthase subunit d, mitochondrial [KO:K02138]
111155772  ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
111147126  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
111151840  ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
111141207  voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
111145442  voltage-dependent anion-selective channel protein 2 [KO:K15040]
111144425  voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
111157245  ADP/ATP translocase 2 [KO:K05863]
111144412  ADP/ATP translocase 1 [KO:K05863]
111157056  ADP/ATP translocase 4 [KO:K05863]
111157123  ADP/ATP translocase 3 [KO:K05863]
111145350  peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
111147305  cytochrome c isoform X1 [KO:K08738]
111142635  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
111141900  LOW QUALITY PROTEIN: uncharacterized protein LOC111141900 [KO:K08738]
111142091  apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
111154673  caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
111161782  proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
111157026  proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
111152671  proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
111142650  LOW QUALITY PROTEIN: proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
111141333  proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
111143461  proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
111159417  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
111140663  proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
111161100  proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
111146069  proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
111151691  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
111143148  proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
111139193  proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
111158705  proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
111161949  proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
111162024  proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
111155299  26S proteasome regulatory subunit 7 [KO:K03061]
111148465  26S proteasome regulatory subunit 4 [KO:K03062]
111161514  26S proteasome regulatory subunit 6B isoform X1 [KO:K03063]
111139115  26S proteasome regulatory subunit 10B [KO:K03064]
111149577  26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
111158036  26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
111148031  26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
111143971  26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
111151573  26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
111156408  26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
111158181  26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
111151359  26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
111144078  26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
111152968  26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
111149610  26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
111161952  26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
111140976  26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
111161550  26S proteasome non-ATPase regulatory subunit 8 isoform X1 [KO:K03031]
111141807  LOW QUALITY PROTEIN: proteasomal ubiquitin receptor ADRM1 [KO:K06691]
111147630  LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
111161402  LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
111158091  serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
111145476  TNF receptor-associated factor 2-like [KO:K03173] [EC:2.3.2.27]
111146100  TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
111147502  mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
111161723  dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
111154883  serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
111140006  serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
111139478  serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
111158002  autophagy-related protein 101 isoform X1 [KO:K19730]
111149764  autophagy-related protein 13 isoform X1 [KO:K08331]
111155388  RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
111149427  activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
111150658  beclin-2 [KO:K08334]
111151520  beclin-1 isoform X1 [KO:K08334]
111155119  phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
111162436  beclin 1-associated autophagy-related key regulator [KO:K17889]
111144899  nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
111142649  phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
111149620  autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
111148489  autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
111145977  WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
111158171  WD repeat domain phosphoinositide-interacting protein 1 [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
elk00190  Oxidative phosphorylation
elk03020  RNA polymerase
elk03022  Basal transcription factors
elk03050  Proteasome
elk04020  Calcium signaling pathway
elk04115  p53 signaling pathway
elk04140  Autophagy - animal
elk04141  Protein processing in endoplasmic reticulum
elk04144  Endocytosis
elk04210  Apoptosis
elk04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

DBGET integrated database retrieval system