KEGG   PATHWAY: pgig05016
Entry
pgig05016                   Pathway                                
Name
Huntington disease - Pteropus giganteus (Indian 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
pgig05016  Huntington disease
pgig05016

Organism
Pteropus giganteus (Indian flying fox) [GN:pgig]
Gene
120598086  CLTA; clathrin light chain A [KO:K04644]
120616541  CLTB; LOW QUALITY PROTEIN: clathrin light chain B [KO:K04645]
120606810  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
120620823  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
120591704  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
120615574  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
120584678  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
120620891  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
120593006  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
120617894  AP2S1; AP-2 complex subunit sigma [KO:K11827]
120582881  HTT; huntingtin [KO:K04533]
120594623  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
120604960  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
120612895  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
120581563  DCTN4; dynactin subunit 4 [KO:K10426]
120584537  DCTN5; dynactin subunit 5 [KO:K10427]
120589056  DCTN6; dynactin subunit 6 [KO:K10428]
120609151  ACTR1A; alpha-centractin [KO:K16575]
120610237  ACTR1B; beta-centractin isoform X1 [KO:K16575]
120603431  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
120611281  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
120602327  DNAH11; dynein heavy chain 11, axonemal isoform X1 [KO:K10408]
120616010  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
120594490  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
120606331  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
120584565  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
120597548  DNAH8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
120619309  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
120586258  DNAH5; dynein heavy chain 5, axonemal isoform X1 [KO:K10408]
120587485  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
120599521  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
120600224  DNAH17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
120589746  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
120593296  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
120612964  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
120600077  dynein intermediate chain 2, axonemal [KO:K11143]
120619480  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
120614805  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
120606832  LOW QUALITY PROTEIN: tubulin alpha-3 chain-like [KO:K07374]
120618200  tubulin alpha-1A chain [KO:K07374]
120618201  TUBA1C; tubulin alpha-1C chain [KO:K07374]
120618203  tubulin alpha-1A chain-like [KO:K07374]
120607368  TUBA8; tubulin alpha-8 chain [KO:K07374]
120587682  tubulin alpha-1B chain-like [KO:K07374]
120587683  tubulin alpha-4A chain [KO:K07374]
120593379  TUBB; tubulin beta chain [KO:K07375]
120603889  TUBB1; tubulin beta-1 chain [KO:K07375]
120615448  TUBB4A; LOW QUALITY PROTEIN: tubulin beta-4A chain [KO:K07375]
120607707  tubulin beta-2A chain [KO:K07375]
120607710  tubulin beta-2A chain-like [KO:K07375]
120599744  TUBB4B; tubulin beta-4B chain isoform X1 [KO:K07375]
120588919  TUBB6; tubulin beta-6 chain [KO:K07375]
120589166  tubulin beta-3 chain [KO:K07375]
120616614  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
120609636  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
120588349  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
120604954  kinesin heavy chain isoform X1 [KO:K10396]
120584120  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
120597184  KIF5C; kinesin heavy chain [KO:K10396]
120613941  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
120605294  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
120584814  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
120596144  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
120598161  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
120587528  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
120618435  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
120590530  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
120588271  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
120587190  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
120600630  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
120611916  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 [KO:K05858] [EC:3.1.4.11]
120611973  PLCB1; LOW QUALITY PROTEIN: 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
120591777  STX1A; syntaxin-1A isoform X1 [KO:K04560]
120599733  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
120599777  GRIN1; glutamate receptor ionotropic, NMDA 1 [KO:K05208]
120613742  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
120582429  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
120589628  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
120581606  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
120613443  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
120612223  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
120599657  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
120603646  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
120586185  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
120605931  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
120584293  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
120588111  REST; RE1-silencing transcription factor [KO:K09222]
120597918  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
120619722  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
120581992  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
120608742  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
120589664  POLR2A; LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
120588110  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
120617328  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
120593442  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
120583689  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
120614829  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
120600849  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
120592927  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
120592493  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
120591631  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
120605221  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
120602971  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
120620322  LOW QUALITY PROTEIN: CREB-binding protein [KO:K04498] [EC:2.3.1.48]
120603899  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
120587251  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
120615800  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
120605605  TBPL2; TATA box-binding protein-like 2 isoform X1 [KO:K03120]
120618243  TATA-box-binding protein [KO:K03120]
120602177  SP1; transcription factor Sp1 [KO:K04684]
120587583  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
120592160  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
120615385  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
120606645  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
120601151  CREB3L2; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
120612863  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
120602391  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
120582783  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
120621474  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
120609383  TFAM; transcription factor A, mitochondrial [KO:K11830]
120601173  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
120598754  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
120598834  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
120588135  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
120622911  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
120611437  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
120581575  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
120582354  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
120608177  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
120599487  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
120621057  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
120612750  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
120613229  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
120589680  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
120606950  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
120617900  BBC3; LOW QUALITY PROTEIN: bcl-2-binding component 3 [KO:K10132]
120584746  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
120588577  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
120598742  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
120613848  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial-like [KO:K03944]
120581414  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
120616482  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
120602254  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
120604950  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
120601258  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
120602938  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
120602939  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6-like [KO:K03950]
120598765  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
120605755  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
120607301  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
120592183  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
120584540  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
120615433  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
120611623  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
120606599  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
120619622  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
120601134  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
120587522  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X1 [KO:K03959]
120586679  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
120597142  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
120612925  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
120618472  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
120609201  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
120595681  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
120620248  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
120605511  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
120587568  NDUFS1; LOW QUALITY PROTEIN: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
120584202  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
120607415  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
120608171  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
120593298  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
120586301  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
120583687  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
120584743  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
120613219  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
120590396  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
120586318  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
120585752  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
120584198  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
120621994  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
120617223  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
120588814  cytochrome c1, heme protein, mitochondrial [KO:K00413]
120582316  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
120584555  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
120620319  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
120605463  cytochrome b-c1 complex subunit 7 [KO:K00417]
120616506  cytochrome b-c1 complex subunit 8 [KO:K00418]
120610685  cytochrome b-c1 complex subunit 8-like [KO:K00418]
120601887  cytochrome b-c1 complex subunit 9 [KO:K00419]
120603514  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
120622928  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
120597795  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
120610239  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
120606788  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
120620534  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
120592433  cytochrome c oxidase subunit 6B1 [KO:K02267]
120598319  cytochrome c oxidase subunit 6B2 [KO:K02267]
120595634  cytochrome c oxidase subunit 6C isoform X1 [KO:K02268]
120592495  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
120594945  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
120609699  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
120621725  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
120591688  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
120588028  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
120588365  cytochrome c oxidase subunit 7B2, mitochondrial-like [KO:K02271]
120586049  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
120604925  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
120609017  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
120583693  ATP5F1D; ATP synthase subunit delta, mitochondrial isoform X1 [KO:K02134]
120585021  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
120602187  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
120596836  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
120611266  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
120600221  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
120598636  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
120598886  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
120581285  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
120609565  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
120595502  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
120591624  ADP/ATP translocase 3-like [KO:K05863]
120616243  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
120618453  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
120586046  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
120589516  ADP/ATP translocase 3 [KO:K05863]
120609581  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
120602359  cytochrome c [KO:K08738]
120597089  cytochrome c, testis-specific-like [KO:K08738]
120611595  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
120585783  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
120620763  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
120591738  PSMA2; proteasome subunit alpha type-2 isoform X1 [KO:K02726] [EC:3.4.25.1]
120599350  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
120603909  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
120587318  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
120584985  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
120603239  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
120603477  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
120607873  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
120601686  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
120608950  sperm motility kinase-like [KO:K02735] [EC:3.4.25.1]
120611217  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
120581927  LOW QUALITY PROTEIN: proteasome subunit beta type-2-like [KO:K02734] [EC:3.4.25.1]
120593235  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
120593764  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
120618217  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
120585246  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
120591506  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
120619604  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
120595865  PSMC4; 26S proteasome regulatory subunit 6B isoform X1 [KO:K03063]
120608146  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 6B-like [KO:K03063]
120604417  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
120607312  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
120600289  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
120592898  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
120597146  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
120611182  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
120616070  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
120600023  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
120620915  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
120593970  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
120614162  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
120585233  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
120597178  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
120596357  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 isoform X1 [KO:K03031]
120603906  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
120614997  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
120595736  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
120600114  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
120599784  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
120615779  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
120598851  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
120585826  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
120615974  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
120589825  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
120602133  ATG101; autophagy-related protein 101 [KO:K19730]
120606859  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
120607252  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
120606809  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
120619070  BECN2; beclin-2 [KO:K08334]
120611068  BECN1; beclin-1 isoform X1 [KO:K08334]
120582068  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
120605604  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
120609419  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
120586191  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
120619671  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
120600867  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
120615611  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
120599998  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
Compound
C00025  L-Glutamate
C00076  Calcium cation
C00238  Potassium cation
C01245  D-myo-Inositol 1,4,5-trisphosphate
C01330  Sodium cation
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
Reference
PMID:15459747
  Authors
Landles C, Bates GP
  Title
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series.
  Journal
EMBO Rep 5:958-63 (2004)
DOI:10.1038/sj.embor.7400250
Reference
PMID:17041811
  Authors
Borrell-Pages M, Zala D, Humbert S, Saudou F
  Title
Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.
  Journal
Cell Mol Life Sci 63:2642-60 (2006)
DOI:10.1007/s00018-006-6242-0
Reference
PMID:18951640
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
DOI:10.1016/j.tins.2008.09.004
Reference
PMID:16288298
  Authors
Cattaneo E, Zuccato C, Tartari M
  Title
Normal huntingtin function: an alternative approach to Huntington's disease.
  Journal
Nat Rev Neurosci 6:919-30 (2005)
DOI:10.1038/nrn1806
Reference
PMID:15336977
  Authors
Bezprozvanny I, Hayden MR
  Title
Deranged neuronal calcium signaling and Huntington disease.
  Journal
Biochem Biophys Res Commun 322:1310-7 (2004)
DOI:10.1016/j.bbrc.2004.08.035
Reference
PMID:14526190
  Authors
Sawa A, Tomoda T, Bae BI
  Title
Mechanisms of neuronal cell death in Huntington's disease.
  Journal
Cytogenet Genome Res 100:287-95 (2003)
DOI:10.1159/000072864
Reference
PMID:12372277
  Authors
Ross CA.
  Title
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
  Journal
Neuron 35:819-22 (2002)
DOI:10.1016/S0896-6273(02)00872-3
Reference
PMID:15242639
  Authors
Ross CA
  Title
Huntington's disease: new paths to pathogenesis.
  Journal
Cell 118:4-7 (2004)
DOI:10.1016/j.cell.2004.06.022
Reference
PMID:24739785
  Authors
Coffey ET
  Title
Nuclear and cytosolic JNK signalling in neurons.
  Journal
Nat Rev Neurosci 15:285-99 (2014)
DOI:10.1038/nrn3729
Reference
PMID:19525941
  Authors
Morfini GA, You YM, Pollema SL, Kaminska A, Liu K, Yoshioka K, Bjorkblom B, Coffey ET, Bagnato C, Han D, Huang CF, Banker G, Pigino G, Brady ST
  Title
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin.
  Journal
Nat Neurosci 12:864-71 (2009)
DOI:10.1038/nn.2346
Reference
PMID:26920688
  Authors
Kumar P, Kumar D, Jha SK, Jha NK, Ambasta RK
  Title
Ion Channels in Neurological Disorders.
  Journal
Adv Protein Chem Struct Biol 103:97-136 (2016)
DOI:10.1016/bs.apcsb.2015.10.006
Reference
PMID:25191533
  Authors
Proft J, Weiss N
  Title
Rectifying rectifier channels in Huntington disease.
  Journal
Commun Integr Biol 7:e29410 (2014)
DOI:10.4161/cib.29410
Reference
PMID:15695335
  Authors
Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I
  Title
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
  Journal
Proc Natl Acad Sci U S A 102:2602-7 (2005)
DOI:10.1073/pnas.0409402102
Reference
PMID:29427371
  Authors
Mackay JP, Nassrallah WB, Raymond LA
  Title
Cause or compensation?-Altered neuronal Ca(2+) handling in Huntington's disease.
  Journal
CNS Neurosci Ther 24:301-310 (2018)
DOI:10.1111/cns.12817
Reference
PMID:11807533
  Authors
Mattson MP.
  Title
Accomplices to neuronal death.
  Journal
Nature 415:377-9 (2002)
DOI:10.1038/415377a
Reference
PMID:17081970
  Authors
McGill JK, Beal MF
  Title
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Journal
Cell 127:465-8 (2006)
DOI:10.1016/j.cell.2006.10.023
Reference
PMID:30941017
  Authors
Intihar TA, Martinez EA, Gomez-Pastor R
  Title
Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1alpha Transcription Factors.
  Journal
Front Cell Neurosci 13:103 (2019)
DOI:10.3389/fncel.2019.00103
Reference
PMID:15996541
  Authors
La Spada AR, Morrison RS
  Title
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
  Journal
Neuron 47:1-3 (2005)
DOI:10.1016/j.neuron.2005.06.023
Reference
PMID:11738471
  Authors
Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
  Journal
Neurochem Int 40:37-52 (2002)
DOI:10.1016/S0197-0186(01)00059-6
Reference
PMID:15996546
  Authors
Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A
  Title
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
  Journal
Neuron 47:29-41 (2005)
DOI:10.1016/j.neuron.2005.06.005
Reference
PMID:11839795
  Authors
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ
  Title
Interaction of Huntington disease protein with transcriptional activator Sp1.
  Journal
Mol Cell Biol 22:1277-87 (2002)
DOI:10.1128/MCB.22.5.1277-1287.2002
Reference
PMID:25270767
  Authors
Vidal RL, Matus S, Bargsted L, Hetz C
  Title
Targeting autophagy in neurodegenerative diseases.
  Journal
Trends Pharmacol Sci 35:583-91 (2014)
DOI:10.1016/j.tips.2014.09.002
Reference
PMID:16522639
  Authors
Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J
  Title
Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1.
  Journal
J Biol Chem 281:14474-85 (2006)
DOI:10.1074/jbc.M600364200
Related
pathway
pgig00190  Oxidative phosphorylation
pgig03020  RNA polymerase
pgig03022  Basal transcription factors
pgig03050  Proteasome
pgig04020  Calcium signaling pathway
pgig04115  p53 signaling pathway
pgig04140  Autophagy - animal
pgig04141  Protein processing in endoplasmic reticulum
pgig04144  Endocytosis
pgig04210  Apoptosis
pgig04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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