KEGG   PATHWAY: pvp05016
Entry
pvp05016                    Pathway                                
Name
Huntington disease - Pteropus vampyrus (large flying fox)
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
pvp05016  Huntington disease
pvp05016

Disease
H00059  Huntington disease
Organism
Pteropus vampyrus (large flying fox) [GN:pvp]
Gene
105288264  DNAL4; dynein light chain 4, axonemal [KO:K10412]
105288270  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
105288316  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
105288463  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
105288552  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
105288781  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
105288878  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
105288933  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
105288974  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
105289192  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
105289274  [KO:K02266]
105289410  CLTCL1; LOW QUALITY PROTEIN: clathrin heavy chain 2 [KO:K04646]
105289473  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
105289485  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
105289628  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
105289933  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
105289976  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
105290018  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
105290020  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
105290037  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
105290042  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
105290070  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
105290124  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
105290165  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X2 [KO:K03949]
105290188  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
105290196  NRF1; nuclear respiratory factor 1 isoform X2 [KO:K11831]
105290575  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
105290624  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
105290643  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
105290744  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
105290810  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
105290990  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
105291036  [KO:K07374]
105291037  [KO:K07374]
105291055  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
105291214  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
105291226  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
105291230  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
105291367  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
105291380  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
105291422  RB1CC1; RB1-inducible coiled-coil protein 1 [KO:K17589]
105291606  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
105291624  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
105291670  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
105291714  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
105291735  DLG4; disks large homolog 4 isoform X3 [KO:K11828]
105291754  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
105291960  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
105292024  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
105292202  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
105292261  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
105292262  PSMA8; proteasome subunit alpha type-7-like isoform X2 [KO:K02731] [EC:3.4.25.1]
105292457  [KO:K04565] [EC:1.15.1.1]
105292546  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
105292615  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
105292672  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
105292810  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
105292883  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X2 [KO:K00914] [EC:2.7.1.137]
105292938  [KO:K02270]
105293163  [KO:K08738]
105293217  [KO:K09047]
105293278  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
105293340  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
105293413  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
105293448  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
105293469  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
105293487  TFAM; transcription factor A, mitochondrial isoform X1 [KO:K11830]
105293528  CLTB; clathrin light chain B [KO:K04645]
105293648  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
105293721  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
105293755  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
105293757  REST; RE1-silencing transcription factor [KO:K09222]
105293916  [KO:K02271]
105294061  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
105294208  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
105294211  GRM5; metabotropic glutamate receptor 5 [KO:K04604]
105294330  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
105294412  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
105294429  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
105294452  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
105294564  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
105294703  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
105294710  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
105294780  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
105294870  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
105294928  [KO:K02271]
105295171  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
105295257  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
105295499  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
105295511  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
105295516  [KO:K05863]
105295633  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
105295843  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
105295986  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
105296085  TUBB1; tubulin beta-1 chain [KO:K07375]
105296096  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
105296192  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
105296219  CLTA; clathrin light chain A [KO:K04644]
105296460  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
105296515  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
105296558  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X2 [KO:K11353]
105296776  [KO:K07375]
105296904  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
105297147  [KO:K00416]
105297234  TUBA8; tubulin alpha-8 chain [KO:K07374]
105297267  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
105297315  BBC3; bcl-2-binding component 3 [KO:K10132]
105297318  AP2S1; AP-2 complex subunit sigma [KO:K11827]
105297346  KLC3; kinesin light chain 3 [KO:K10407]
105297445  GRIA4; glutamate receptor 4 isoform X2 [KO:K05200]
105297536  TUBA1B; tubulin alpha-1B chain isoform X1 [KO:K07374]
105297537  [KO:K07374]
105297584  [KO:K07374]
105297603  [KO:K03968]
105297704  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
105297724  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
105297760  ERN1; LOW QUALITY PROTEIN: serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
105297813  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
105297836  [KO:K02273]
105297846  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
105297863  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
105297932  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
105298025  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
105298051  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
105298127  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
105298131  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X1 [KO:K03959]
105298163  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
105298309  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
105298404  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
105298415  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
105298543  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
105298557  TUBB4B; tubulin beta-4B chain [KO:K07375]
105298564  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
105298593  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
105298613  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
105298737  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
105298800  ATG2B; autophagy-related protein 2 homolog B [KO:K17906]
105298814  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
105298839  [KO:K02263]
105298980  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
105299024  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
105299248  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
105299286  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
105299444  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
105299454  DCTN4; dynactin subunit 4 [KO:K10426]
105299460  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
105299561  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
105299678  STX1A; syntaxin-1A isoform X1 [KO:K04560]
105299689  HIP1; huntingtin-interacting protein 1 [KO:K04559]
105299706  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
105299873  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
105299885  DCTN6; dynactin subunit 6 [KO:K10428]
105299978  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
105300193  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 isoform X2 [KO:K03031]
105300213  MAP3K10; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
105300217  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
105300378  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
105300529  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
105300538  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
105300597  [KO:K00417]
105300719  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
105300789  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X2 [KO:K03957]
105300868  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
105300922  [KO:K00419]
105301070  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
105301083  TUBB4A; tubulin beta-4A chain [KO:K07375]
105301194  [KO:K00411] [EC:7.1.1.8]
105301227  HTT; huntingtin [KO:K04533]
105301261  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
105301308  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
105301309  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
105301411  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
105301482  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
105301572  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
105301667  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
105301679  DCTN5; dynactin subunit 5 [KO:K10427]
105301729  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
105301762  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
105301970  [KO:K00413]
105302106  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
105302219  [KO:K00420]
105302238  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
105302240  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
105302384  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
105302414  [KO:K00414]
105302452  [KO:K04634]
105302549  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X2 [KO:K03948]
105302566  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
105302646  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
105302655  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
105302783  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
105302898  [KO:K02268]
105302901  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
105303098  CREBBP; CREB-binding protein [KO:K04498] [EC:2.3.1.48]
105303190  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
105303219  PSMA2; proteasome subunit alpha type-2 isoform X1 [KO:K02726] [EC:3.4.25.1]
105303324  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 isoform X1 [KO:K03966]
105303396  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
105303403  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
105303717  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
105303723  TBP; TATA-box-binding protein [KO:K03120]
105303837  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
105303843  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
105303918  [KO:K02272]
105304244  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
105304445  KLC1; kinesin light chain 1 [KO:K10407]
105304499  [KO:K02266]
105304627  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
105304852  TUBB3; tubulin beta-3 chain [KO:K07375]
105304910  [KO:K02267]
105304972  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
105305094  [KO:K04565] [EC:1.15.1.1]
105305214  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
105305394  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944]
105305404  BECN2; beclin-2 [KO:K08334]
105305449  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
105305502  [KO:K02263]
105305546  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
105305558  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
105305666  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
105305799  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X2 [KO:K03937]
105305805  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
105305962  AP2A2; AP-2 complex subunit alpha-2 [KO:K11824]
105305985  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
105306053  [KO:K00415]
105306057  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
105306136  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
105306187  [KO:K10408]
105306274  BECN1; beclin-1 isoform X1 [KO:K08334]
105306339  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
105306353  TUBB6; tubulin beta-6 chain [KO:K07375]
105306380  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
105306407  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
105306431  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
105306436  DCTN3; dynactin subunit 3 isoform X4 [KO:K10425]
105306466  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
105306559  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
105306564  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
105306643  [KO:K02267]
105306673  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
105306745  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
105306771  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
105306782  ACTR1B; beta-centractin isoform X1 [KO:K16575]
105306783  [KO:K02265]
105306845  [KO:K02264]
105306854  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
105306947  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
105306976  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
105307005  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
105307010  ATG101; autophagy-related protein 101 [KO:K19730]
105307108  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
105307210  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
105307242  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
105307329  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
105307381  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
105307450  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
105307464  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
105307466  [KO:K02731] [EC:3.4.25.1]
105307507  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
105307510  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
105307700  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
105307710  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
105307875  [KO:K02270]
105307890  [KO:K02270]
105307906  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
105308002  [KO:K05863]
105308138  TUBB; tubulin beta chain [KO:K07375]
105308386  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
105308414  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
105308512  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
105308580  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
105308883  [KO:K02271]
105308921  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
105308931  ACTR10; actin-related protein 10 [KO:K16576]
105309091  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
105309288  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
105309466  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
105309658  ACTR1A; alpha-centractin [KO:K16575]
105309810  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
105310149  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
105310590  [KO:K07375]
105311019  [KO:K00418]
105311066  [KO:K03954]
111734519  [KO:K03939]
111742157  [KO:K03944]
111745811  [KO:K04565] [EC:1.15.1.1]
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
pvp00190  Oxidative phosphorylation
pvp03020  RNA polymerase
pvp03022  Basal transcription factors
pvp03050  Proteasome
pvp04020  Calcium signaling pathway
pvp04115  p53 signaling pathway
pvp04140  Autophagy - animal
pvp04141  Protein processing in endoplasmic reticulum
pvp04144  Endocytosis
pvp04210  Apoptosis
pvp04724  Glutamatergic synapse
KO pathway
ko05016   

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