KEGG   PATHWAY: shr05016
Entry
shr05016                    Pathway                                
Name
Huntington disease - Sarcophilus harrisii (Tasmanian devil)
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
shr05016  Huntington disease
shr05016

Organism
Sarcophilus harrisii (Tasmanian devil) [GN:shr]
Gene
100926895  CLTA; clathrin light chain A isoform X1 [KO:K04644]
100933527  CLTB; clathrin light chain B isoform X2 [KO:K04645]
100920538  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
100935396  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
100934530  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
100924206  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
100921341  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
100913959  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
100930280  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
116423036  AP2S1; AP-2 complex subunit sigma [KO:K11827]
100930812  HTT; huntingtin isoform X1 [KO:K04533]
100917792  DCTN1; dynactin subunit 1 isoform X9 [KO:K04648]
100934636  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
100914013  DCTN3; dynactin subunit 3 [KO:K10425]
100935346  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
100930245  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
100923016  DCTN6; dynactin subunit 6 [KO:K10428]
100917201  ACTR1A; alpha-centractin [KO:K16575]
100919728  ACTR1B; beta-centractin isoform X1 [KO:K16575]
100925558  ACTR10; actin-related protein 10 [KO:K16576]
100916192  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
100923394  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
100922026  DNAH12; dynein heavy chain 12, axonemal isoform X2 [KO:K10408]
100917337  DNAH17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
100935107  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
100926140  DNAH14; dynein heavy chain 14, axonemal [KO:K10408]
100933744  DNAH8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
100921501  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
100930983  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
100927585  DNAH3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
100927390  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
100922472  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
100914911  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
100934605  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
100913750  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
100927173  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
100918331  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
100914220  DNAL4; dynein light chain 4, axonemal [KO:K10412]
100932522  tubulin alpha-1D chain isoform X2 [KO:K07374]
100932786  tubulin alpha-4A chain [KO:K07374]
100920562  tubulin alpha-8 chain [KO:K07374]
100914278  LOW QUALITY PROTEIN: tubulin alpha chain [KO:K07374]
100923972  TUBA1C; tubulin alpha-1C chain [KO:K07374]
100927621  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
105750812  LOW QUALITY PROTEIN: tubulin alpha-1A chain [KO:K07374]
100920490  tubulin alpha-1A chain [KO:K07374]
100923223  tubulin alpha-1C chain-like [KO:K07374]
116420883  tubulin alpha-3 chain [KO:K07374]
100932736  TUBB3; tubulin beta-3 chain [KO:K07375]
100930400  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
100920771  TUBB4B; tubulin beta-4B chain [KO:K07375]
100913653  tubulin beta-2B chain [KO:K07375]
100929714  TUBB4A; tubulin beta-4A chain [KO:K07375]
100934059  tubulin beta-2A chain [KO:K07375]
100932679  TUBB6; tubulin beta-6 chain [KO:K07375]
100918219  TUBB; tubulin beta chain isoform X2 [KO:K07375]
100930815  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100928480  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
100924267  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100931867  KIF5A; kinesin heavy chain isoform X3 [KO:K10396]
100916485  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
100921508  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
100929070  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
100924072  KLC2; kinesin light chain 2 [KO:K10407]
100924347  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
100925884  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
100927072  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
100931580  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
100922757  caspase-3-like [KO:K02187] [EC:3.4.22.56]
100922500  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100913241  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
100916183  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634]
100922938  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
100934049  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
100934311  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
100929015  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
100914393  STX1A; syntaxin-1A [KO:K04560]
100933192  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
100922600  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X3 [KO:K05208]
100926323  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100918532  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X7 [KO:K04958]
100930883  DLG4; disks large homolog 4 isoform X3 [KO:K11828]
100924175  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
100927884  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
100925736  GRIA3; glutamate receptor 3 isoform X2 [KO:K05199]
100929056  GRIA4; glutamate receptor 4 isoform X5 [KO:K05200]
100930830  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
100932840  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
100923371  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
100915507  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
100921808  REST; RE1-silencing transcription factor isoform X1 [KO:K09222]
100929520  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
100928076  RCOR1; REST corepressor 1 [KO:K11829]
100913322  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
100927352  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
100926404  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
100921014  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
100918939  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
100935294  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
100927834  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
100923711  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
100913810  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
100933932  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
116419918  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
100930889  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
100935194  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
100934810  BDNF; brain-derived neurotrophic factor isoform X2 [KO:K04355]
100921187  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
100926283  CREBBP; CREB-binding protein isoform X2 [KO:K04498] [EC:2.3.1.48]
100931108  TAF4B; transcription initiation factor TFIID subunit 4B isoform X2 [KO:K03129]
100925832  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
100920337  LOW QUALITY PROTEIN: transcription initiation factor TFIID subunit 4-like [KO:K03129]
100915353  TBP; TATA-box-binding protein isoform X1 [KO:K03120]
100925485  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
100934885  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
100930024  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
100913327  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
100926416  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
100913890  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
100916355  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100917654  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100923082  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
100922067  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
100931691  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X5 [KO:K07202]
100934154  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
100932137  TFAM; transcription factor A, mitochondrial [KO:K11830]
100933595  NRF1; nuclear respiratory factor 1 [KO:K11831]
100920450  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100922476  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
100923607  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
100917835  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
100916680  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100931013  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
100933291  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
100922473  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
116423037  BBC3; LOW QUALITY PROTEIN: bcl-2-binding component 3 [KO:K10132]
13826446  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
13826439  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
13826438  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
13826443  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
13826441  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
13826442  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
13826444  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100934645  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100928428  NDUFV2; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100919401  LOW QUALITY PROTEIN: uncharacterized protein LOC100919401 [KO:K03944]
100919666  uncharacterized protein LOC100919666 [KO:K03944]
100933088  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100929783  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
100929856  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
116419640  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100924322  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
100919967  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
100918785  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100913375  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100933750  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100915617  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
100924549  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100927317  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
100927182  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100922703  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100922855  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100930539  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100927339  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100918876  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100934778  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
111719323  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
100913747  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100926601  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
116420693  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100917145  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100915928  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
100914378  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
100922998  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
111721451  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100930936  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
100932609  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100933792  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100922533  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
100918238  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100929004  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100928800  NDUFC2; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100931806  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
100924299  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
100918297  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
100916627  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
100920165  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
13826445  CYTB; cytochrome b [KO:K00412]
100923048  cytochrome c1, heme protein, mitochondrial [KO:K00413]
100919227  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
100935369  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100933846  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
100928777  cytochrome b-c1 complex subunit 7 [KO:K00417]
100934825  cytochrome b-c1 complex subunit 8 [KO:K00418]
100933375  cytochrome b-c1 complex subunit 9 [KO:K00419]
100931111  cytochrome b-c1 complex subunit 10 [KO:K00420]
13826436  COX3; cytochrome c oxidase subunit III [KO:K02262]
13826440  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
13826435  COX2; cytochrome c oxidase subunit II [KO:K02261]
100924710  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
100922691  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X5 [KO:K02263]
100928744  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
100919989  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
100918528  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
111719270  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100922461  cytochrome c oxidase subunit 6B1 [KO:K02267]
100934493  cytochrome c oxidase subunit 6C [KO:K02268]
100921435  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100930775  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
100928083  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100922983  cytochrome c oxidase subunit 7A1, mitochondrial-like [KO:K02270]
100932664  cytochrome c oxidase subunit 7B, mitochondrial isoform X1 [KO:K02271]
100933884  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
100932575  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100921543  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100920745  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100934419  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100930145  ATP5F1E; LOW QUALITY PROTEIN: ATP synthase subunit epsilon, mitochondrial [KO:K02135]
13826434  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100916300  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100926225  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100914229  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X2 [KO:K02128]
100915508  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100930357  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
100923940  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100931914  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
13826437  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100927813  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100920408  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
100923123  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
100920060  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100929945  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
116422319  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
100925120  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
100919671  cytochrome c 2 [KO:K08738]
111721251  cytochrome c [KO:K08738]
100932636  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
100931481  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
100917209  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
100930249  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100921548  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
100923039  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100929199  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
100921704  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
100934809  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100925826  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
100934267  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100915465  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100918897  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100924826  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100924260  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100915095  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100921265  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100920574  PSMC2; 26S proteasome regulatory subunit 7 isoform X1 [KO:K03061]
100932408  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100921684  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100923300  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
100917535  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
100922728  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
100931153  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
100926409  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100925706  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100924888  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
100920479  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
100927917  PSMD6; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100923392  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100923814  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100920478  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
100933723  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X2 [KO:K03030]
100915675  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100921743  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X2 [KO:K06691]
100919883  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
100932009  MAP3K10; LOW QUALITY PROTEIN: mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
100935398  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100934749  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
100917439  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
100926110  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
100931480  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
100934149  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
100934448  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
100931777  ATG101; autophagy-related protein 101 [KO:K19730]
100930382  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
100933709  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
100914667  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
100931761  BECN1; beclin-1 isoform X1 [KO:K08334]
100919089  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
100924525  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
100923107  NRBF2; nuclear receptor-binding factor 2 [KO:K21246]
100924620  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
100914858  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
100917633  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
100920269  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
100931931  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
shr00190  Oxidative phosphorylation
shr03020  RNA polymerase
shr03022  Basal transcription factors
shr03050  Proteasome
shr04020  Calcium signaling pathway
shr04115  p53 signaling pathway
shr04140  Autophagy - animal
shr04141  Protein processing in endoplasmic reticulum
shr04144  Endocytosis
shr04210  Apoptosis
shr04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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