KEGG   PATHWAY: clud05016
Entry
clud05016                   Pathway                                
Name
Huntington disease - Canis lupus dingo (dingo)
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
clud05016  Huntington disease
clud05016

Organism
Canis lupus dingo (dingo) [GN:clud]
Gene
112650419  CLTA; clathrin light chain A isoform X1 [KO:K04644]
112651884  CLTB; clathrin light chain B isoform X1 [KO:K04645]
112655549  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
112676984  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
112646165  HIP1; huntingtin-interacting protein 1 isoform X3 [KO:K04559]
112663247  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
112645128  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
112655334  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
112662705  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
112645255  AP2S1; AP-2 complex subunit sigma isoform X1 [KO:K11827]
112653709  HTT; huntingtin isoform X1 [KO:K04533]
112664238  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
112677980  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
112669882  LOW QUALITY PROTEIN: dynactin subunit 2-like [KO:K10424]
112668807  dynactin subunit 2-like [KO:K10424]
112652314  dynactin subunit 2-like [KO:K10424]
112649686  DCTN3; dynactin subunit 3 [KO:K10425]
112651349  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
112679043  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
112641060  DCTN6; dynactin subunit 6 [KO:K10428]
112670797  ACTR1B; beta-centractin isoform X1 [KO:K16575]
112672247  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
112657113  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
112655255  HAP1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
112656666  DNAH7; LOW QUALITY PROTEIN: dynein axonemal heavy chain 7 [KO:K10408]
112647351  DNAH9; dynein axonemal heavy chain 9 isoform X2 [KO:K10408]
112647422  DNAH2; dynein axonemal heavy chain 2 isoform X1 [KO:K10408]
112648831  DNAH14; dynein axonemal heavy chain 14 isoform X1 [KO:K10408]
112672874  DNAH17; dynein axonemal heavy chain 17 isoform X1 [KO:K10408]
112640276  DNAH3; dynein axonemal heavy chain 3 isoform X1 [KO:K10408]
112664294  DNAH6; LOW QUALITY PROTEIN: dynein axonemal heavy chain 6 [KO:K10408]
112641530  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
112662682  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
112670558  DNAH11; LOW QUALITY PROTEIN: dynein axonemal heavy chain 11 [KO:K10408]
112665880  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
112665952  DNAH12; dynein axonemal heavy chain 12 isoform X1 [KO:K10408]
112676451  DNAH10; LOW QUALITY PROTEIN: dynein axonemal heavy chain 10 [KO:K10408]
112642379  DNALI1; axonemal dynein light intermediate polypeptide 1 isoform X1 [KO:K10410]
112649679  DNAI1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
112672752  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
112657112  DNAL1; dynein axonemal light chain 1 isoform X1 [KO:K10411]
112667707  DNAL4; dynein axonemal light chain 4 isoform X1 [KO:K10412]
112667043  tubulin alpha chain-like isoform X1 [KO:K07374]
112656371  tubulin alpha-1B chain isoform X1 [KO:K07374]
112656372  TUBA4A; tubulin alpha-4A chain isoform X1 [KO:K07374]
112678286  tubulin alpha-1B chain isoform X1 [KO:K07374]
112678287  tubulin alpha-1A chain [KO:K07374]
112678288  tubulin alpha-1C chain isoform X1 [KO:K07374]
112659064  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
112668951  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
112677006  tubulin alpha-3 chain-like [KO:K07374]
112677007  tubulin alpha-3 chain [KO:K07374]
112677497  TUBB4B; tubulin beta-4B chain isoform X1 [KO:K07375]
112668213  tubulin beta chain-like isoform X1 [KO:K07375]
112646852  TUBB3; tubulin beta-3 chain [KO:K07375]
112659313  TUBB2A; tubulin beta-2A chain isoform X4 [KO:K07375]
112648004  LOW QUALITY PROTEIN: tubulin beta chain [KO:K07375]
112648102  TUBB6; tubulin beta-6 chain [KO:K07375]
112674192  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
112641255  TUBB; tubulin beta chain [KO:K07375]
112666377  TUBB4A; tubulin beta-4A chain [KO:K07375]
112645941  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
112659577  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
112671968  MAPK8; mitogen-activated protein kinase 8 isoform X3 [KO:K04440] [EC:2.7.11.24]
112677975  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
112670360  KIF5B; kinesin-1 heavy chain [KO:K10396]
112649490  KIF5C; kinesin heavy chain [KO:K10396]
112645307  KLC3; kinesin light chain 3 isoform X2 [KO:K10407]
112641611  KLC4; kinesin light chain 4 [KO:K10407]
112657137  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
112678904  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
112656364  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
112675231  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
112642041  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
112644686  guanine nucleotide-binding protein G(q) subunit alpha isoform X2 [KO:K04634]
112659377  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
112673608  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X2 [KO:K05858] [EC:3.1.4.11]
112673609  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
112676127  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X5 [KO:K05858] [EC:3.1.4.11]
112646152  STX1A; syntaxin-1A [KO:K04560]
112677513  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X4 [KO:K04849]
112677485  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
112665422  GRIN2B; glutamate receptor ionotropic, NMDA 2B isoform X1 [KO:K05210]
112662581  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
112647484  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
112651162  GRIA1; glutamate receptor 1 isoform X2 [KO:K05197]
112654419  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
112649055  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
112671422  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
112673931  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
112652633  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
112663471  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
112642978  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 isoform X1 [KO:K05003]
112652141  REST; RE1-silencing transcription factor [KO:K09222]
112675561  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
112657969  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
112658381  HDAC2; histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
112659842  HDAC1; histone deacetylase 1 isoform X2 [KO:K06067] [EC:3.5.1.98]
112647455  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
112652287  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
112660458  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
112652413  POLR2D; DNA-directed RNA polymerase II subunit RPB4 isoform X2 [KO:K03012]
112666170  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X2 [KO:K03013]
112668238  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X2 [KO:K03014]
112659371  POLR2G; DNA-directed RNA polymerase II subunit RPB7 isoform X1 [KO:K03015]
112662719  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
112643613  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
112663254  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
112646186  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
112662095  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
112671754  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
112658086  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
112667789  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
112667202  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
112640446  CREB-binding protein isoform X2 [KO:K04498] [EC:2.3.1.48]
112647939  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
112674219  TAF4; LOW QUALITY PROTEIN: transcription initiation factor TFIID subunit 4 [KO:K03129]
112644152  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
112658264  TATA-box-binding protein isoform X1 [KO:K03120]
112667184  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
112656471  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
112647736  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
112670436  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
112650165  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
112663293  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
112666968  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
112670641  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
112654092  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
112661459  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
112651159  TFAM; transcription factor A, mitochondrial [KO:K11830]
112675080  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
112661655  SOD1; superoxide dismutase [Cu-Zn] isoform X1 [KO:K04565] [EC:1.15.1.1]
112644383  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
112657019  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
112659973  probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
112660097  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
112651953  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
112674373  GPX5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
112642454  GPX7; glutathione peroxidase 7 isoform X2 [KO:K00432] [EC:1.11.1.9]
112665783  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
112674374  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
112669314  UCP1; mitochondrial brown fat uncoupling protein 1 isoform X3 [KO:K08769]
112647438  TP53; cellular tumor antigen p53 [KO:K04451]
112645177  BAX; apoptosis regulator BAX [KO:K02159]
112645247  BBC3; bcl-2-binding component 3 [KO:K10132]
112647056  NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
112659341  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
112648058  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
112669177  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
112649282  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
112663986  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
112655613  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
112669712  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
112658832  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
112653127  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
112651067  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
125752483  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
112671733  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
112677988  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X1 [KO:K03948]
112674847  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
112675526  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112659729  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112643457  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112675938  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112649746  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
112675011  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
118354020  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
112657429  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
112666700  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
112666407  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
112677158  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952]
112669059  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
112670006  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
112640237  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
112651654  acyl carrier protein, mitochondrial-like [KO:K03955]
112666355  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 isoform X4 [KO:K03956]
112654563  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
112666789  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
118351720  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
112646040  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
118355324  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
118355555  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
112650992  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
112649336  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
112656283  NDUFB3; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
112677535  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 isoform X2 [KO:K03960]
112662850  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
112644265  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial-like [KO:K03961]
112650486  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
112678755  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6-like [KO:K03962]
112666636  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
112672201  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
112662129  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
112640558  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
112648012  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10-like [KO:K03966]
112673263  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
112656793  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
112643024  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
112663336  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
112651294  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
112646084  NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial-like [KO:K03937]
112642341  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 isoform X1 [KO:K03938]
112662703  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
112666186  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
112659312  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X3 [KO:K03941] [EC:7.1.1.2]
112667486  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial-like [KO:K03941] [EC:7.1.1.2]
112669293  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
112642121  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
112662906  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
112660061  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
112675119  succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial-like [KO:K00235] [EC:1.3.5.1]
112643036  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
112671340  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
112645623  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
112662205  cytochrome c1, heme protein, mitochondrial [KO:K00413]
112665750  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
112640264  cytochrome b-c1 complex subunit 2, mitochondrial isoform X2 [KO:K00415]
112669654  cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
112642556  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
112650722  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
112650058  cytochrome b-c1 complex subunit 8 isoform X1 [KO:K00418]
112676792  cytochrome b-c1 complex subunit 9 isoform X2 [KO:K00419]
112666200  cytochrome b-c1 complex subunit 10 [KO:K00420]
112646773  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
112673765  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
112669019  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
112675573  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
112670808  cytochrome c oxidase subunit 5B, mitochondrial isoform X1 [KO:K02265]
112669031  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
112640146  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
112676672  cytochrome c oxidase subunit 6A1, mitochondrial isoform X1 [KO:K02266]
112667822  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
112655679  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
112644991  cytochrome c oxidase subunit 6B2 [KO:K02267]
112660066  cytochrome c oxidase subunit 6B1-like [KO:K02267]
112665643  cytochrome c oxidase subunit 6B1 [KO:K02267]
112643623  cytochrome c oxidase subunit 6B1 isoform X1 [KO:K02267]
112657639  cytochrome c oxidase subunit 6B1-like [KO:K02267]
112678400  cytochrome c oxidase subunit 6C [KO:K02268]
112643625  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
112658567  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
112664458  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
118355483  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
112671885  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
112652117  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
112643473  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
112650945  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
112664558  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
118352292  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
112678421  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
112653522  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
112675990  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
112671697  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
112669591  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
125753053  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
112659430  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
112671942  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
112653719  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
112647792  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
112661675  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
112678005  ATP5F1B; ATP synthase subunit beta, mitochondrial isoform X5 [KO:K02133] [EC:7.1.2.2]
112666177  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
112674194  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
112640661  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
112655310  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
112667173  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
112668135  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
112662982  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
118355186  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
112648876  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
112655416  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
118350644  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
112647980  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112670682  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112654881  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112655980  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112659988  ATP synthase subunit d, mitochondrial [KO:K02138]
112668567  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112641957  ATP synthase subunit d, mitochondrial-like [KO:K02138]
125754599  ATP synthase subunit d, mitochondrial-like [KO:K02138]
125754631  ATP synthase subunit d, mitochondrial-like [KO:K02138]
112672785  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
112661712  ATP5PO; ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
112668013  ATP synthase subunit O, mitochondrial-like [KO:K02137]
112645053  ATP synthase subunit O, mitochondrial-like [KO:K02137]
112678736  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
112661929  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
118350204  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
112671433  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
112651940  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
112656506  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
112654613  voltage-dependent anion-selective channel protein 3-like [KO:K15041]
112673224  voltage-dependent anion-selective channel protein 3-like [KO:K15041]
112649317  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
112651963  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
112675217  SLC25A4; ADP/ATP translocase 1 isoform X1 [KO:K05863]
112642775  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
112651452  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
112670591  cytochrome c [KO:K08738]
112678206  LOW QUALITY PROTEIN: cytochrome c-like [KO:K08738]
112653556  LOW QUALITY PROTEIN: cytochrome c-like [KO:K08738]
112654697  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
112659987  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
112657128  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
112651142  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
112661294  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
112662212  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
112647941  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
112674222  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
112640716  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
112657136  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
112667667  PSMA1; proteasome subunit alpha type-1 isoform X2 [KO:K02725] [EC:3.4.25.1]
112662342  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
112669358  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
112641493  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
112678369  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
112645056  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
112653675  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
112675844  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
125754355  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
112657247  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
112647521  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
112677182  PSMB7; proteasome subunit beta type-7 isoform X2 [KO:K02739] [EC:3.4.25.1]
112655657  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
112658404  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
112642411  PSMB2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
112657343  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
112658266  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
112663964  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
112661487  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
112656874  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
112659172  26S proteasome regulatory subunit 4-like [KO:K03062]
112645470  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
112657073  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
112663330  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
112671786  26S proteasome regulatory subunit 6A-like [KO:K03065]
112652916  PSMC5; 26S proteasome regulatory subunit 8 [KO:K03066]
112662784  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
112669877  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
112655117  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
112676500  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
112652946  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
112655095  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
112666018  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
112646563  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
112663553  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
112663973  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
112674684  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
112645523  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
112674229  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
112669413  26S proteasome complex subunit SEM1 [KO:K10881]
112674881  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
112645461  MAP3K10; mitogen-activated protein kinase kinase kinase 10 isoform X1 [KO:K04418] [EC:2.7.11.25]
112652886  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
112677465  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
112644173  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
112666418  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
112678102  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
112676397  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
112647287  ULK2; serine/threonine-protein kinase ULK2 isoform X2 [KO:K08269] [EC:2.7.11.1]
112667239  ATG101; autophagy-related protein 101 [KO:K19730]
112663299  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
112678441  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
112663298  AMBRA1; LOW QUALITY PROTEIN: activating molecule in BECN1-regulated autophagy protein 1 [KO:K17985]
112655399  BECN1; beclin-1 isoform X1 [KO:K08334]
112648727  BECN2; beclin-2 isoform X1 [KO:K08334]
112661148  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
112657096  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
112651329  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
112647820  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
112657714  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
112659323  ATG2A; LOW QUALITY PROTEIN: autophagy-related protein 2 homolog A [KO:K17906]
112646361  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X2 [KO:K17908]
112653018  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
clud00190  Oxidative phosphorylation
clud03020  RNA polymerase
clud03022  Basal transcription factors
clud03050  Proteasome
clud04020  Calcium signaling pathway
clud04115  p53 signaling pathway
clud04140  Autophagy - animal
clud04141  Protein processing in endoplasmic reticulum
clud04144  Endocytosis
clud04210  Apoptosis
clud04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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