KEGG   PATHWAY: ssc05016
Entry
ssc05016                    Pathway                                
Name
Huntington disease - Sus scrofa (pig)
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
ssc05016  Huntington disease
ssc05016

Disease
H00059  Huntington disease
Organism
Sus scrofa (pig) [GN:ssc]
Gene
100037274  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
100037985  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100037988  ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
100037989  COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
100037990  COX7C; cytochrome c oxidase subunit 7C, mitochondrial precursor [KO:K02272]
100037991  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100037992  PSMB7; proteasome subunit beta type-7 precursor [KO:K02739] [EC:3.4.25.1]
100038000  COX7A2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100038022  COX6B; cytochrome c oxidase subunit 6B1 [KO:K02267]
100038031  COX8H; COX8H protein [KO:K02273]
100048954  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
100125545  [KO:K03120]
100127131  [KO:K07374]
100127359  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
100134963  ATP5E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
100134983  TUBB1; tubulin beta-1 chain [KO:K07375]
100135051  TUBA1A; tubulin alpha-1A chain [KO:K07374]
100142688  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
100142689  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100151951  TUBA4A; tubulin alpha-4A chain [KO:K07374]
100151990  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
100152232  COX6A1; cytochrome c oxidase subunit VIa polypeptide 1 [KO:K02266]
100152612  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
100152679  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
100153507  TUBB2B; tubulin beta-2B chain [KO:K07375]
100153828  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100153923  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100154091  GPX6; glutathione peroxidase 6 precursor [KO:K00432] [EC:1.11.1.9]
100154130  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100154148  PSMC6; 26S protease regulatory subunit 10B [KO:K03064]
100154319  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
100154408  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
100154426  TBPL2; TATA box-binding protein-like protein 2 isoform X6 [KO:K03120]
100154443  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100154530  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
100154817  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100155138  [KO:K07374]
100155139  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100155274  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
100155621  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
100155666  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
100155687  UQCR10; cytochrome b-c1 complex subunit 9 [KO:K00419]
100155804  RCOR1; REST corepressor 1 [KO:K11829]
100156016  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
100156075  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
100156170  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
100156226  EP300; LOW QUALITY PROTEIN: histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
100156288  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100156327  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
100156478  DNAH8; LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal [KO:K10408]
100156521  DNAL1; dynein light chain 1, axonemal [KO:K10411]
100156619  ACTR1A; alpha-centractin [KO:K16575]
100156790  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
100156879  [KO:K00414]
100156967  [KO:K02264]
100156985  [KO:K03007]
100157156  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100157157  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
100157315  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
100157442  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
100157621  UQCRB; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
100157641  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
100157738  NRBF2; nuclear receptor-binding factor 2 isoform X3 [KO:K21246]
100157866  DNAH7; LOW QUALITY PROTEIN: dynein heavy chain 7, axonemal [KO:K10408]
100157880  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100157935  [KO:K02273]
100158003  [KO:K07374]
100158185  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100170131  CYCS; cytochrome c [KO:K08738]
100187581  CLTA; clathrin light chain A isoform X1 [KO:K04644]
100271929  CLTC; clathrin heavy chain 1 [KO:K04646]
100312966  DCTN6; dynactin subunit 6 [KO:K10428]
100312976  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100462716  ATG2B; autophagy-related protein 2 homolog B [KO:K17906]
100462717  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
100462758  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
100462759  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
100510930  [KO:K07374]
100510948  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100510978  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100511241  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
100511252  ACTR1B; LOW QUALITY PROTEIN: beta-centractin [KO:K16575]
100511777  DNAH2; LOW QUALITY PROTEIN: dynein heavy chain 2, axonemal [KO:K10408]
100512253  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100512454  PSMC3; 26S protease regulatory subunit 6A isoform X1 [KO:K03065]
100512594  STX1A; LOW QUALITY PROTEIN: syntaxin-1A [KO:K04560]
100512624  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
100512673  BECN2; beclin-2 [KO:K08334]
100513030  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100513262  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
100513283  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
100513566  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
100513629  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100513739  PSMA8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
100513840  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
100513895  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100514033  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
100514408  AP2S1; AP-2 complex subunit sigma isoform 1 [KO:K11827]
100514655  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100514865  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
100515069  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100515483  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100515635  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
100515661  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
100516047  SLC1A3; excitatory amino acid transporter 1 isoform 1 [KO:K05614]
100516128  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
100516161  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
100516293  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
100516352  TUBB4B; tubulin beta-4B chain [KO:K07375]
100516402  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
100516480  [KO:K02271]
100516581  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
100516606  [KO:K02270]
100516663  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
100516779  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100517196  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100517203  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
100517403  BBC3; bcl-2-binding component 3 isoform X3 [KO:K10132]
100517408  [KO:K02271]
100517563  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
100517634  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100517815  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100518016  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100518253  TUBA8; tubulin alpha-8 chain [KO:K07374]
100518397  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
100518564  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100518913  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
100519249  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
100519295  [KO:K08738]
100519335  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
100519691  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
100519700  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X3 [KO:K17985]
100519870  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100519871  [KO:K02128]
100519957  KIF5A; kinesin heavy chain isoform X1 [KO:K10396] [EC:5.6.1.3]
100519996  GRIA3; glutamate receptor 3 isoform X2 [KO:K05199]
100520115  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100520250  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
100520304  DNAL4; dynein light chain 4, axonemal [KO:K10412]
100520312  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
100520418  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X5 [KO:K04431] [EC:2.7.12.2]
100520468  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
100520754  REST; RE1-silencing transcription factor [KO:K09222]
100520950  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
100521453  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
100521478  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
100521540  KLC2; kinesin light chain 2 isoform X2 [KO:K10407]
100521614  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
100521736  MAPK10; mitogen-activated protein kinase 10 isoform X3 [KO:K04440] [EC:2.7.11.24]
100521815  DCTN3; dynactin subunit 3 isoform X2 [KO:K10425]
100521945  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
100522678  [KO:K00411] [EC:7.1.1.8]
100522725  [KO:K02273]
100522753  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
100522775  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
100522783  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100522842  [KO:K08738]
100522979  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100523278  PSMD11; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100523423  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100523598  HIP1; LOW QUALITY PROTEIN: huntingtin-interacting protein 1 [KO:K04559]
100523621  KLC1; kinesin light chain 1 isoform X7 [KO:K10407]
100523751  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
100524239  [KO:K00420]
100524308  [KO:K00418]
100524394  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100524560  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
100524613  [KO:K00415]
100524622  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100524641  DCTN5; dynactin subunit 5 [KO:K10427]
100524676  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
100524798  DNAH6; dynein heavy chain 6, axonemal isoform X1 [KO:K10408]
100524873  [KO:K00416]
100524886  GRIA4; glutamate receptor 4 isoform X3 [KO:K05200]
100525083  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X2 [KO:K03959]
100525148  PLCB3; LOW QUALITY PROTEIN: 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
100525255  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
100525371  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
100525780  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
100525860  CLTB; clathrin light chain B isoform X1 [KO:K04645]
100525869  [KO:K00413]
100579141  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100620215  CREBBP; CREB-binding protein [KO:K04498] [EC:2.3.1.48]
100620235  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
100620420  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100620457  AP2B1; AP-2 complex subunit beta [KO:K11825]
100620543  DNAH11; dynein heavy chain 11, axonemal isoform X1 [KO:K10408]
100620619  ACTR10; actin-related protein 10 [KO:K16576]
100620737  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
100620809  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
100621006  [KO:K09222]
100621221  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
100621347  [KO:K08738]
100621417  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
100621969  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100622102  KIF5C; kinesin heavy chain [KO:K10396] [EC:5.6.1.3]
100622110  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100622245  ATP5J; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
100622444  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100622482  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
100622699  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
100622769  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
100622950  GRIA2; glutamate receptor 2 isoform X2 [KO:K05198]
100623248  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
100623333  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
100623355  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
100623583  TUBB6; LOW QUALITY PROTEIN: tubulin beta-6 chain [KO:K07375]
100623647  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
100623873  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100623921  DNAH5; LOW QUALITY PROTEIN: dynein heavy chain 5, axonemal [KO:K10408]
100624067  [KO:K02263]
100624095  DNAI2; dynein intermediate chain 2, axonemal isoform X1 [KO:K11143]
100624335  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100624738  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
100624785  [KO:K07375]
100625977  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
100626022  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100626043  DCTN4; dynactin subunit 4 [KO:K10426]
100626077  DLG4; disks large homolog 4 isoform X6 [KO:K11828]
100626250  [KO:K02266]
100626274  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100626452  TAF4; LOW QUALITY PROTEIN: transcription initiation factor TFIID subunit 4 [KO:K03129]
100626738  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100626761  AP2M1; AP-2 complex subunit mu [KO:K11826]
100627068  SLC1A2; excitatory amino acid transporter 2 isoform X2 [KO:K05613]
100627568  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100627780  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
100628241  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
100736562  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
100736584  TUBB4A; tubulin beta-4A chain [KO:K07375]
100736934  HAP1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
100737040  POLR2A; LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
100737214  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
100737579  POLR2D; DNA-directed RNA polymerase II subunit RPB4 isoform X1 [KO:K03012]
100737726  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100738073  DNAH3; LOW QUALITY PROTEIN: dynein heavy chain 3, axonemal [KO:K10408]
100739619  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
102157770  [KO:K06691]
102157773  [KO:K02268]
102158866  KLC3; kinesin light chain 3 [KO:K10407]
102160861  ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
102161751  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
102163805  DNAH17; LOW QUALITY PROTEIN: dynein heavy chain 17, axonemal [KO:K10408]
102164336  CACNA1B; LOW QUALITY PROTEIN: voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
110257420  [KO:K02263]
110257483  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
110259740  TBP; TATA-box-binding protein [KO:K03120]
110260663  ATG101; autophagy-related protein 101 [KO:K19730]
110260986  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
110261055  [KO:K02267]
396575  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
396598  GPX3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
396609  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
396610  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
396633  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
396920  GPX5; epididymal secretory glutathione peroxidase precursor [KO:K00432] [EC:1.11.1.9]
397001  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
397010  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
397013  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha [KO:K07202]
397014  HTT; huntingtin [KO:K04533]
397036  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
397043  PSMC5; 26S protease regulatory subunit 8 [KO:K03066]
397058  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
397244  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
397276  TP53; cellular tumor antigen p53 [KO:K04451]
397279  TFAM; transcription factor A, mitochondrial precursor [KO:K11830]
397403  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
397440  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
397454  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
397495  BDNF; brain-derived neurotrophic factor precursor [KO:K04355]
397651  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
397659  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
397671  PPARG; peroxisome proliferator-activated receptor gamma [KO:K08530]
399685  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial precursor [KO:K02270]
403155  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
414403  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform 1 [KO:K03017]
414412  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235] [EC:1.3.5.1]
448979  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
492822  COX5B; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265]
497063  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
497064  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
497065  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
503700  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
574054  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
595105  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
595132  KIF5B; kinesin-1 heavy chain [KO:K10396] [EC:5.6.1.3]
654405  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
733576  BECN1; beclin-1 [KO:K08334]
733583  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
733594  TUBA1B; tubulin alpha-1B chain [KO:K07374]
733605  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
733678  ATP5O; ATP synthase subunit O, mitochondrial precursor [KO:K02137]
733686  TUBB; tubulin beta chain [KO:K07375]
767625  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
780433  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
808501  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
808502  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
808503  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
808504  COX2; cytochrome c oxidase subunit II [KO:K02261]
808505  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
808506  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
808507  COX3; cytochrome c oxidase subunit III [KO:K02262]
808508  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
808509  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
808510  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
808511  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
808512  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
808513  CYTB; cytochrome b [KO:K00412]
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
ssc00190  Oxidative phosphorylation
ssc03020  RNA polymerase
ssc03022  Basal transcription factors
ssc03050  Proteasome
ssc04020  Calcium signaling pathway
ssc04115  p53 signaling pathway
ssc04140  Autophagy - animal
ssc04141  Protein processing in endoplasmic reticulum
ssc04144  Endocytosis
ssc04210  Apoptosis
ssc04724  Glutamatergic synapse
KO pathway
ko05016   

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