KEGG   PATHWAY: pcoq05016
Entry
pcoq05016                   Pathway                                
Name
Huntington disease - Propithecus coquereli (Coquerel's sifaka)
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
pcoq05016  Huntington disease
pcoq05016

Disease
H00059  Huntington disease
Organism
Propithecus coquereli (Coquerel's sifaka) [GN:pcoq]
Gene
105804976  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
105805182  DNAL4; dynein light chain 4, axonemal [KO:K10412]
105805267  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
105805275  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
105805288  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
105805900  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
105806035  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
105806167  PLCB1; LOW QUALITY PROTEIN: 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
105806182  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
105806237  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
105806291  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
105806434  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
105806589  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
105806701  [KO:K02271]
105806914  ATP5J; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
105807023  [KO:K02266]
105807050  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
105807057  STX1A; syntaxin-1A [KO:K04560]
105807087  [KO:K04559]
105807261  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
105807267  [KO:K02264]
105807308  [KO:K02136]
105807356  AP2M1; AP-2 complex subunit mu [KO:K11826]
105807372  [KO:K09047]
105807375  [KO:K08738]
105807458  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
105807521  DNAH12; LOW QUALITY PROTEIN: dynein heavy chain 12, axonemal [KO:K10408]
105807577  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
105807678  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
105807685  REST; RE1-silencing transcription factor [KO:K09222]
105807933  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
105807947  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
105807952  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
105808078  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
105808189  PSMA8; proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
105808190  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
105808222  GRM5; metabotropic glutamate receptor 5 [KO:K04604]
105808354  [KO:K02265]
105808355  ACTR1B; beta-centractin [KO:K16575]
105808520  ATP5C1; ATP synthase subunit gamma, mitochondrial [KO:K02136]
105808587  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
105808623  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
105808667  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
105808789  TUBA4A; tubulin alpha-4A chain isoform X1 [KO:K07374]
105808883  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
105808886  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
105808983  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
105809025  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
105809316  BAX; apoptosis regulator BAX [KO:K02159]
105809322  [KO:K02267]
105809407  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
105809466  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
105809556  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
105809719  [KO:K03946]
105809747  [KO:K02268]
105809797  [KO:K00417]
105810108  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
105810111  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
105810116  DNAH14; LOW QUALITY PROTEIN: dynein heavy chain 14, axonemal [KO:K10408]
105810204  [KO:K00419]
105810258  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
105810555  [KO:K08738]
105810742  [KO:K02272]
105810750  [KO:K11825]
105810895  [KO:K00415]
105810898  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
105810924  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
105810930  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
105811004  HDAC2; LOW QUALITY PROTEIN: histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
105811066  PSMC3; 26S protease regulatory subunit 6A [KO:K03065]
105811072  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
105811079  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
105811088  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
105811104  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
105811110  [KO:K02270]
105811230  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
105811257  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
105811286  [KO:K00420]
105811289  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
105811699  IFT57; LOW QUALITY PROTEIN: intraflagellar transport protein 57 homolog [KO:K04638]
105811799  [KO:K02270]
105811880  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
105811892  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
105811984  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
105812168  [KO:K02263]
105812318  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
105812321  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
105812396  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
105812491  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
105812509  [KO:K08738]
105812563  [KO:K00411] [EC:7.1.1.8]
105812612  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
105812624  RCOR1; REST corepressor 1 [KO:K11829]
105812700  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
105812706  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
105813050  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
105813122  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
105813404  [KO:K00413]
105813564  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
105813610  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
105813663  CLTC; clathrin heavy chain 1 [KO:K04646]
105813718  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
105813828  AP2B1; AP-2 complex subunit beta [KO:K11825]
105814072  [KO:K00416]
105814447  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
105814456  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
105814556  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
105814588  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
105814632  [KO:K02128]
105814705  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
105814731  [KO:K02730] [EC:3.4.25.1]
105814740  DCTN6; dynactin subunit 6 [KO:K10428]
105814773  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
105814822  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
105814986  ATG2B; autophagy-related protein 2 homolog B [KO:K17906]
105815072  [KO:K02266]
105815191  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
105815301  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
105815384  TUBA1A; tubulin alpha-1A chain isoform X1 [KO:K07374]
105815399  [KO:K07374]
105815409  [KO:K07374]
105815495  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
105815498  [KO:K07374]
105815572  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
105815609  [KO:K08738]
105815718  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
105815768  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
105815811  TUBA8; tubulin alpha-8 chain [KO:K07374]
105815885  [KO:K07374]
105815999  [KO:K04440] [EC:2.7.11.24]
105816029  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
105816045  [KO:K00418]
105816062  [KO:K07375]
105816116  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
105816211  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
105816214  [KO:K02270]
105816235  [KO:K02267]
105816385  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
105816408  KIF5A; kinesin heavy chain isoform 5A isoform X1 [KO:K10396]
105816458  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
105816542  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
105816545  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
105816548  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
105816683  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
105816719  PSMD1; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
105816836  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
105817103  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
105817257  [KO:K03030]
105817283  TUBB; tubulin beta chain [KO:K07375]
105817329  GRIA2; glutamate receptor 2 [KO:K05198]
105817487  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
105817539  AP2S1; AP-2 complex subunit sigma [KO:K11827]
105817554  [KO:K02136]
105817577  BBC3; bcl-2-binding component 3 [KO:K10132]
105817696  KLC3; kinesin light chain 3 [KO:K10407]
105817755  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
105817917  ATG101; autophagy-related protein 101 [KO:K19730]
105817968  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
105817990  [KO:K02270]
105818043  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
105818062  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
105818168  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
105818236  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
105818525  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
105818541  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
105818550  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
105818686  TUBB4B; tubulin beta-4B chain [KO:K07375]
105818690  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
105818700  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
105818713  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
105818745  KIF5B; kinesin-1 heavy chain [KO:K10396]
105818751  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
105818837  CLTCL1; LOW QUALITY PROTEIN: clathrin heavy chain 2 [KO:K04646]
105818911  HTT; huntingtin isoform X1 [KO:K04533]
105819019  PPARG; peroxisome proliferator-activated receptor gamma [KO:K08530]
105819043  [KO:K02273]
105819128  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
105819291  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
105819386  DLG4; disks large homolog 4 [KO:K11828]
105819390  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
105819395  TP53; cellular tumor antigen p53 [KO:K04451]
105819421  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
105819461  POLR2A; DNA-directed RNA polymerase II subunit RPB1 isoform X1 [KO:K03006] [EC:2.7.7.6]
105819477  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
105819542  PSMD14; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
105819624  [KO:K07374]
105819681  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
105819705  DNAI1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409]
105819753  DNAL1; dynein light chain 1, axonemal [KO:K10411]
105819851  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
105820021  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
105820072  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
105820126  CLTA; clathrin light chain A [KO:K04644]
105820147  DCTN3; dynactin subunit 3 [KO:K10425]
105820245  BECN1; beclin-1 isoform X1 [KO:K08334]
105820356  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
105820430  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
105820583  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
105820593  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
105820609  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
105820631  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
105820645  [KO:K07375]
105820727  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
105820827  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
105820831  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
105820870  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
105821053  TFAM; transcription factor A, mitochondrial [KO:K11830]
105821070  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
105821432  DNAH8; LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal [KO:K10408]
105821478  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
105821498  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
105821573  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
105821574  DCTN4; dynactin subunit 4 [KO:K10426]
105821892  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
105821904  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
105821915  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
105822085  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
105822174  GRIA4; glutamate receptor 4 [KO:K05200]
105822197  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
105822335  TBP; TATA-box-binding protein isoform X1 [KO:K03120]
105822336  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
105822425  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
105822459  KIF5C; kinesin heavy chain isoform 5C [KO:K10396]
105822510  CLTB; clathrin light chain B [KO:K04645]
105822665  HAP1; huntingtin-associated protein 1 [KO:K04647]
105822845  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
105822860  [KO:K11828]
105822913  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
105823056  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
105823339  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
105823587  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
105823643  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
105823905  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
105823945  CREB1; cyclic AMP-responsive element-binding protein 1 [KO:K05870]
105823955  PSMC5; 26S protease regulatory subunit 8 [KO:K03066]
105823972  [KO:K02263]
105824001  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
105824035  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105824092  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
105824114  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
105824126  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
105824299  ACTR1A; alpha-centractin [KO:K16575]
105824308  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
105824355  [KO:K00414]
105824433  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
105824696  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
105824734  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
105824794  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
105824798  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
105824812  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
105824864  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
105824883  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
105825008  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
105825063  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105825121  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
105825486  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
105825488  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
105825547  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
105825563  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
105825671  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
105825831  ACTR10; actin-related protein 10 [KO:K16576]
105825838  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
105825847  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
105825876  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
105825918  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
105826075  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
105826090  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
105826139  [KO:K04849]
105826189  [KO:K02271]
105826255  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
105826272  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
105826365  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
105826377  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
105826451  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
105826559  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
105826732  NRF1; nuclear respiratory factor 1 [KO:K11831]
105826811  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
105826892  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
105827070  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
105827162  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
105827201  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
105827242  [KO:K07375]
105827297  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
105827315  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
105827373  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
105827391  KLC2; kinesin light chain 2 [KO:K10407]
105827410  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
105827413  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
105827532  [KO:K00237]
105827572  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
105827580  [KO:K02263]
6458115  COX2; cytochrome c oxidase subunit II [KO:K02261]
6458116  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
6458117  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
6458118  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
6458119  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
6458120  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
6458121  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
6458122  CYTB; cytochrome b [KO:K00412]
6458123  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
6458124  COX3; cytochrome c oxidase subunit III [KO:K02262]
6458125  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
6458126  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
6458127  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
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
pcoq00190  Oxidative phosphorylation
pcoq03020  RNA polymerase
pcoq03022  Basal transcription factors
pcoq03050  Proteasome
pcoq04020  Calcium signaling pathway
pcoq04115  p53 signaling pathway
pcoq04140  Autophagy - animal
pcoq04141  Protein processing in endoplasmic reticulum
pcoq04144  Endocytosis
pcoq04210  Apoptosis
pcoq04724  Glutamatergic synapse
KO pathway
ko05016   

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