KEGG   PATHWAY: dle05016
Entry
dle05016                    Pathway                                
Name
Huntington disease - Delphinapterus leucas (beluga whale)
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
dle05016  Huntington disease
dle05016

Disease
H00059  Huntington disease
Organism
Delphinapterus leucas (beluga whale) [GN:dle]
Gene
111162636  [KO:K02266]
111162798  [KO:K00419]
111162874  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
111162930  [KO:K02272]
111162959  [KO:K07374]
111163019  TUBB6; tubulin beta-6 chain [KO:K07375]
111163074  [KO:K07374]
111163102  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
111163109  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
111163116  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
111163197  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
111163665  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
111163814  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
111163853  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
111164113  [KO:K03957]
111164181  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
111164367  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
111164612  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
111164824  [KO:K06067] [EC:3.5.1.98]
111164829  [KO:K06067] [EC:3.5.1.98]
111164871  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
111164902  HTT; huntingtin isoform X2 [KO:K04533]
111165123  [KO:K00413]
111165265  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
111165370  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
111165456  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
111165552  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
111165617  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
111165653  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
111165862  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
111165889  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
111165948  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
111166011  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
111166070  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
111166115  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
111166133  [KO:K00420]
111166137  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
111166150  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
111166195  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
111166314  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
111166341  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
111166353  BECN1; beclin-1 isoform X1 [KO:K08334]
111166412  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
111166424  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
111166580  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
111166656  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
111166859  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
111166971  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
111166990  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
111167181  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
111167191  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
111167564  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
111167565  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
111167604  NRBF2; nuclear receptor-binding factor 2 [KO:K21246]
111167760  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
111168028  TBP; TATA-box-binding protein [KO:K03120]
111168047  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
111168108  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
111168155  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
111168206  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
111168252  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
111168263  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
111168438  [KO:K00432] [EC:1.11.1.9]
111168440  GPX6; LOW QUALITY PROTEIN: glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
111168714  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
111168836  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
111168878  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
111168952  [KO:K08530]
111169059  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
111169129  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
111169199  [KO:K03010] [EC:2.7.7.6]
111169240  REST; RE1-silencing transcription factor isoform X1 [KO:K09222]
111169265  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
111169295  [KO:K09222]
111169718  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
111169730  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
111169822  CLTB; clathrin light chain B isoform X1 [KO:K04645]
111169895  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
111170121  [KO:K07374]
111170282  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
111170285  [KO:K02273]
111170304  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
111170343  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
111170348  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
111170487  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
111170494  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
111170529  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
111170669  TUBB; tubulin beta chain [KO:K07375]
111171000  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
111171050  AP2B1; LOW QUALITY PROTEIN: AP-2 complex subunit beta [KO:K11825]
111171524  [KO:K02271]
111171679  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
111171723  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
111171726  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
111171760  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
111171769  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
111171848  [KO:K07374]
111171851  [KO:K07374]
111171923  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
111171993  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
111172029  [KO:K02273]
111172168  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
111172361  ACTR1A; alpha-centractin [KO:K16575]
111172389  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
111172449  [KO:K03943] [EC:7.1.1.2]
111172557  TFAM; transcription factor A, mitochondrial [KO:K11830]
111172744  KIF5C; kinesin heavy chain [KO:K10396]
111172793  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
111172892  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
111172911  [KO:K08738]
111173062  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
111173114  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
111173116  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
111173130  ATG14; beclin 1-associated autophagy-related key regulator isoform X2 [KO:K17889]
111173152  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
111173226  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
111173227  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
111173238  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
111173316  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
111173380  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
111173401  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
111173410  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
111173475  DCTN6; dynactin subunit 6 [KO:K10428]
111173555  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
111173600  [KO:K00237]
111173776  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
111173834  KIF5A; kinesin heavy chain isoform X2 [KO:K10396]
111173835  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
111173848  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
111173862  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
111173931  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
111173942  SP1; transcription factor Sp1 [KO:K04684]
111173965  [KO:K02268]
111173978  ATG101; autophagy-related protein 101 [KO:K19730]
111174031  [KO:K08738]
111174050  [KO:K07374]
111174051  [KO:K07374]
111174052  [KO:K07374]
111174169  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
111174197  [KO:K08738]
111174220  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
111174254  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
111174313  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
111174397  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
111174428  [KO:K00414]
111174571  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
111174575  [KO:K08738]
111174833  [KO:K02270]
111174841  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
111175110  [KO:K21246]
111175514  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
111175568  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
111175599  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
111175620  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
111175766  [KO:K00416]
111175818  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
111175930  [KO:K08738]
111175937  [KO:K03960]
111175981  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
111176031  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
111176051  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
111176104  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
111176166  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
111176274  DNAL1; dynein light chain 1, axonemal [KO:K10411]
111176582  TUBA8; tubulin alpha-8 chain isoform X2 [KO:K07374]
111176583  [KO:K07374]
111176645  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
111176787  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
111176963  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
111176980  TUBB4B; tubulin beta-4B chain [KO:K07375]
111176991  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
111177009  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
111177231  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
111177253  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
111177424  BECN2; beclin-2 [KO:K08334]
111177666  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
111177826  [KO:K02264]
111177835  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
111177869  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
111177953  [KO:K02268]
111177962  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
111177988  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
111178120  KIF5B; kinesin-1 heavy chain [KO:K10396]
111178277  [KO:K02271]
111178333  [KO:K02135]
111178464  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
111178524  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
111178547  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
111178574  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
111178771  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
111178841  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
111178932  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
111179014  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
111179428  [KO:K02263]
111179575  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
111179686  [KO:K07375]
111179780  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
111179820  BDNF; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
111180038  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
111180078  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
111180225  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
111180384  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
111180389  [KO:K02268]
111180406  [KO:K00417]
111180543  [KO:K08738]
111180596  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
111180640  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
111180693  BBC3; bcl-2-binding component 3 [KO:K10132]
111180701  AP2S1; AP-2 complex subunit sigma [KO:K11827]
111180743  KLC3; kinesin light chain 3 [KO:K10407]
111180858  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
111180860  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
111180900  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
111180964  [KO:K00411] [EC:7.1.1.8]
111180991  [KO:K02267]
111181038  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
111181212  [KO:K02270]
111181213  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
111181221  [KO:K03958]
111181225  [KO:K02267]
111181308  TUBB2A; tubulin beta-2A chain [KO:K07375]
111181312  [KO:K07375]
111181329  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
111181484  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
111181486  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
111181538  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
111181753  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
111182011  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
111182027  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
111182042  STX1A; syntaxin-1A isoform X1 [KO:K04560]
111182105  [KO:K02266]
111182238  DCTN5; dynactin subunit 5 [KO:K10427]
111182239  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
111182267  [KO:K00415]
111182272  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
111182430  [KO:K00418]
111182441  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
111182541  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111182582  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
111182616  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
111182623  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
111182644  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
111182750  [KO:K10408]
111182751  [KO:K10408]
111182847  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
111182905  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
111182969  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
111183100  DCTN4; dynactin subunit 4 [KO:K10426]
111183108  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
111183125  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
111183230  [KO:K02270]
111183343  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
111183542  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
111183554  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
111183653  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
111183658  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
111183714  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
111183789  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
111183812  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
111183826  [KO:K02273]
111183849  POLR2G; DNA-directed RNA polymerase II subunit RPB7 isoform X1 [KO:K03015]
111183956  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
111183961  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
111183977  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
111183981  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
111183987  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
111184191  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
111184329  [KO:K02263]
111184500  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
111184910  [KO:K03968]
111184956  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
111185164  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
111185248  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
111185662  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
111185755  TP53; cellular tumor antigen p53 [KO:K04451]
111185756  POLR2A; DNA-directed RNA polymerase II subunit RPB1 isoform X1 [KO:K03006] [EC:2.7.7.6]
111185761  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
111185804  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
111185881  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
111185965  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
111186169  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
111186230  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
111186395  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
111186412  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
111186470  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
111186637  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
111186724  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
111186725  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
111186772  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
111186806  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
111186817  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
111186827  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
111186839  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
111186887  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
111187099  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
111187104  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
111187265  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
111187469  ACTR1B; beta-centractin [KO:K16575]
111187475  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
111187511  [KO:K02265]
111187821  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
111187864  [KO:K15041]
111188007  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
111188103  CLTA; clathrin light chain A isoform X1 [KO:K04644]
31412977  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
31412980  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
31412985  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
31412987  COX2; cytochrome c oxidase subunit II [KO:K02261]
31412988  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
31412989  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
31412990  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
31412993  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
31412994  CYTB; cytochrome b [KO:K00412]
31413000  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
31413001  COX3; cytochrome c oxidase subunit III [KO:K02262]
31413004  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
31413006  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [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
dle00190  Oxidative phosphorylation
dle03020  RNA polymerase
dle03022  Basal transcription factors
dle03050  Proteasome
dle04020  Calcium signaling pathway
dle04115  p53 signaling pathway
dle04140  Autophagy - animal
dle04141  Protein processing in endoplasmic reticulum
dle04144  Endocytosis
dle04210  Apoptosis
dle04724  Glutamatergic synapse
KO pathway
ko05016   

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