KEGG   PATHWAY: dro05016
Entry
dro05016                    Pathway                                
Name
Huntington disease - Desmodus rotundus (common vampire bat)
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
dro05016  Huntington disease
dro05016

Organism
Desmodus rotundus (common vampire bat) [GN:dro]
Gene
112304385  CLTA; clathrin light chain A isoform X1 [KO:K04644]
112320358  CLTB; clathrin light chain B isoform X1 [KO:K04645]
112316442  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
112314856  HIP1; huntingtin-interacting protein 1 [KO:K04559]
112317081  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
112310049  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
112298620  AP2B1; LOW QUALITY PROTEIN: AP-2 complex subunit beta [KO:K11825]
112307925  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
112312929  AP2S1; AP-2 complex subunit sigma isoform X1 [KO:K11827]
112316670  HTT; huntingtin [KO:K04533]
112302952  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
112319022  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
112304444  DCTN3; dynactin subunit 3 [KO:K10425]
112319573  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
112304772  DCTN5; dynactin subunit 5 [KO:K10427]
112306771  DCTN6; dynactin subunit 6 [KO:K10428]
112300360  ACTR1A; alpha-centractin [KO:K16575]
112315190  ACTR1B; beta-centractin [KO:K16575]
112311341  ACTR10; actin-related protein 10 [KO:K16576]
112298857  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
112301281  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
112308449  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
112308525  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
112298919  DNAH17; LOW QUALITY PROTEIN: dynein heavy chain 17, axonemal [KO:K10408]
112304822  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
112303059  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
112306932  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
112318876  DNAH14; dynein heavy chain 14, axonemal isoform X1 [KO:K10408]
112302197  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
112307638  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
112310449  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
112320953  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
112301142  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
112299529  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
112304534  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
112298891  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
112311725  DNAL1; dynein light chain 1, axonemal [KO:K10411]
112321875  DNAL4; dynein light chain 4, axonemal [KO:K10412]
112321272  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
112301806  tubulin alpha-3 chain [KO:K07374]
112318638  tubulin alpha-1A chain [KO:K07374]
112318639  TUBA1B; tubulin alpha-1B chain [KO:K07374]
112311601  TUBA8; tubulin alpha-8 chain [KO:K07374]
112301743  tubulin alpha-3 chain-like [KO:K07374]
112307747  tubulin alpha-4A chain [KO:K07374]
112318375  tubulin alpha-1C chain [KO:K07374]
112307926  tubulin alpha-1D chain-like [KO:K07374]
112296747  TUBB3; LOW QUALITY PROTEIN: tubulin beta-3 chain [KO:K07375]
112303881  TUBB1; tubulin beta-1 chain [KO:K07375]
112306332  TUBB4B; tubulin beta-4B chain [KO:K07375]
112312455  TUBB4A; tubulin beta-4A chain [KO:K07375]
112302104  TUBB; tubulin beta chain [KO:K07375]
112322846  TUBB6; tubulin beta-6 chain [KO:K07375]
112297548  TUBB2B; tubulin beta-2B chain [KO:K07375]
112297549  tubulin beta-2A chain [KO:K07375]
112296379  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
112305558  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
112317841  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
112317665  KIF5B; kinesin-1 heavy chain [KO:K10396]
112319077  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
112313129  KIF5C; kinesin heavy chain [KO:K10396]
112311242  KLC1; kinesin light chain 1 [KO:K10407]
112312892  KLC3; kinesin light chain 3 [KO:K10407]
112302291  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
112317086  KLC2; kinesin light chain 2 [KO:K10407]
112320657  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
112307787  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
112306749  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
112316028  GRM5; LOW QUALITY PROTEIN: metabotropic glutamate receptor 5 [KO:K04604]
112300359  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
112304122  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
112311437  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
112304255  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
112317151  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
112314874  STX1A; syntaxin-1A [KO:K04560]
112306604  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
112306277  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
112318404  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
112309625  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
112308630  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
112319547  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
112312372  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
112310477  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
112314285  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
112304011  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
112321023  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
112303351  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
112314761  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
112322213  REST; RE1-silencing transcription factor [KO:K09222]
112301633  SIN3A; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
112311460  RCOR1; REST corepressor 1 [KO:K11829]
112299318  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
112297024  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
112308505  POLR2A; DNA-directed RNA polymerase II subunit RPB1 isoform X1 [KO:K03006] [EC:2.7.7.6]
112322189  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
112296656  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
112313098  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
112312635  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
112321687  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
112317340  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
112305134  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
112320103  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
112317192  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
112314922  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
112315576  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
112319104  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
112303343  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
112321623  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
112298356  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
112322803  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
112303988  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
112297176  TBP; TATA-box-binding protein isoform X1 [KO:K03120]
112311996  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
112297070  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
112318338  SP1; transcription factor Sp1 [KO:K04684]
112307981  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
112304550  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
112303463  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
112299769  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
112312688  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
112316383  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
112306927  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
112316938  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha [KO:K07202]
112301119  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
112305569  TFAM; transcription factor A, mitochondrial [KO:K11830]
112299740  NRF1; nuclear respiratory factor 1 [KO:K11831]
112305484  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
112297154  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
112309490  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
112312010  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
112319560  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
112315290  GPX8; probable glutathione peroxidase 8 [KO:K00432] [EC:1.11.1.9]
112314658  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
112308825  TP53; cellular tumor antigen p53 [KO:K04451]
112310132  BAX; apoptosis regulator BAX [KO:K02159]
112312833  BBC3; bcl-2-binding component 3 [KO:K10132]
17046640  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
17046643  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
17046637  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
17046627  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
17046631  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
17046641  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
17046628  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
112317410  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
112322679  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
112306093  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
112303408  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
112310507  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
112318106  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
112305021  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
112321282  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
112319170  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
112319834  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
112299939  NDUFA5; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
112321783  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
112305159  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
112306425  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
112313094  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
112307837  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
112304755  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
112312492  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
112321926  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
112305228  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
112311794  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
112299902  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
112307804  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
112320640  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
112307720  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
112304586  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
112301387  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
112300388  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
112315325  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
112298294  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
112322448  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
112307774  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
112298899  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
112303379  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
112315253  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
112299378  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
112321034  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
112312661  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
112317128  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
112312496  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
112316078  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
112321014  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
112299141  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
112298888  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
112314355  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
112319551  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
112296783  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
17046636  CYTB; cytochrome b [KO:K00412]
112315682  cytochrome c1, heme protein, mitochondrial [KO:K00413]
112309855  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
112304767  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
112314624  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
112315581  cytochrome b-c1 complex subunit 7 [KO:K00417]
112318142  cytochrome b-c1 complex subunit 8 [KO:K00418]
112310709  cytochrome b-c1 complex subunit 9 [KO:K00419]
112312414  cytochrome b-c1 complex subunit 10 [KO:K00420]
17046617  COX3; cytochrome c oxidase subunit III [KO:K02262]
17046649  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
17046633  COX2; cytochrome c oxidase subunit II [KO:K02261]
112304202  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
112300797  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
112301898  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
112315210  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
112310631  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
112304878  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
112313525  cytochrome c oxidase subunit 6B2 [KO:K02267]
112320945  cytochrome c oxidase subunit 6B1-like [KO:K02267]
112318743  cytochrome c oxidase subunit 6B1-like [KO:K02267]
112320116  cytochrome c oxidase subunit 6B1 [KO:K02267]
112303723  cytochrome c oxidase subunit 6B1-like [KO:K02267]
112304781  cytochrome c oxidase subunit 6C-like [KO:K02268]
112315524  cytochrome c oxidase subunit 6C [KO:K02268]
112297964  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
112319300  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
112320156  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
112316645  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
112317931  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
112307507  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
112317280  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
112322763  ATP5F1A; LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial [KO:K02132]
112319107  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
112322744  ATP5F1C; ATP synthase subunit gamma, mitochondrial [KO:K02136]
112312379  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
112300834  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
112300842  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
112303928  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
17046618  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
112313721  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
112318265  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
112305812  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
112309410  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
112316280  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
112298792  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
112305628  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
112306721  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
17046642  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
112318103  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
112303041  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 2-like [KO:K15040]
112305359  VDAC2; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 2 [KO:K15040]
112321447  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
112310580  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
112313387  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
112301943  ADP/ATP translocase 3-like [KO:K05863]
112312642  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
112306713  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
112305493  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
112306993  cytochrome c [KO:K08738]
112320658  cytochrome c-like [KO:K08738]
112322221  cytochrome c-like [KO:K08738]
112321814  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
112299456  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
112296520  PSMA6; LOW QUALITY PROTEIN: proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
112302715  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
112306047  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
112303886  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
112322942  PSMA8; proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
112314015  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
112303431  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
112311343  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
112308535  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
112306265  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
112316536  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
112299348  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
112301454  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
112297308  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
112314197  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
112303293  PSMC2; LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 7 [KO:K03061]
112311308  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
112320204  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
112311346  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
112303423  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
112316274  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
112307999  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
112307664  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
112316357  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
112310800  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
112316281  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
112308494  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
112301262  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
112300781  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
112321353  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 13-like [KO:K03039]
112317219  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
112314086  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
112322238  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
112320217  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
112303932  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
112305530  SEM1; LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
112320192  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
112316288  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
112306448  TRAF2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
112297079  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
112305131  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
112299108  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
112310681  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
112308452  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
112318619  ATG101; autophagy-related protein 101 [KO:K19730]
112303238  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
112320833  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
112303248  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
112298578  BECN1; beclin-1 [KO:K08334]
112318767  BECN2; beclin-2 [KO:K08334]
112309598  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
112311368  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
112305448  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
112322978  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
112317076  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
112311845  autophagy-related protein 2 homolog B [KO:K17906]
112319592  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
112316590  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [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
dro00190  Oxidative phosphorylation
dro03020  RNA polymerase
dro03022  Basal transcription factors
dro03050  Proteasome
dro04020  Calcium signaling pathway
dro04115  p53 signaling pathway
dro04140  Autophagy - animal
dro04141  Protein processing in endoplasmic reticulum
dro04144  Endocytosis
dro04210  Apoptosis
dro04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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