KEGG   PATHWAY: cfa05016
Entry
cfa05016                    Pathway                                
Name
Huntington disease - Canis lupus familiaris (dog)
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
cfa05016  Huntington disease
cfa05016

Organism
Canis lupus familiaris (dog) [GN:cfa]
Gene
100856559  CLTA; clathrin light chain A isoform X4 [KO:K04644]
489104  CLTB; clathrin light chain B isoform X1 [KO:K04645]
477568  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
480578  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
489814  HIP1; huntingtin-interacting protein 1 isoform X2 [KO:K04559]
475994  AP2A2; AP-2 complex subunit alpha-2 isoform X4 [KO:K11824]
484375  AP2A1; AP-2 complex subunit alpha-1 isoform X2 [KO:K11824]
480605  AP2B1; AP-2 complex subunit beta isoform X2 [KO:K11825]
607139  AP2M1; AP-2 complex subunit mu [KO:K11826]
476427  AP2S1; AP-2 complex subunit sigma [KO:K11827]
479074  HTT; huntingtin isoform X2 [KO:K04533]
483106  DCTN1; dynactin subunit 1 isoform X4 [KO:K04648]
474415  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
474547  LOW QUALITY PROTEIN: dynactin subunit 2-like [KO:K10424]
611471  LOW QUALITY PROTEIN: dynactin subunit 2-like [KO:K10424]
474750  DCTN3; dynactin subunit 3 isoform X2 [KO:K10425]
479326  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
479804  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
475600  DCTN6; dynactin subunit 6 isoform X1 [KO:K10428]
403791  ACTR1A; alpha-centractin [KO:K16575]
611680  ACTR1B; beta-centractin isoform X2 [KO:K16575]
480337  ACTR10; actin-related protein 10 [KO:K16576]
607751  HAP1; huntingtin-associated protein 1 isoform X2 [KO:K04647]
476598  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
489981  DNAH3; dynein heavy chain 3, axonemal isoform X8 [KO:K10408]
489480  DNAH2; dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
488452  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
606983  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
475774  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
489507  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
482346  DNAH11; LOW QUALITY PROTEIN: dynein heavy chain 11, axonemal [KO:K10408]
484715  DNAH12; LOW QUALITY PROTEIN: dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
474895  DNAH8; dynein heavy chain 8, axonemal isoform X5 [KO:K10408]
486244  DNAH10; dynein heavy chain 10, axonemal isoform X8 [KO:K10408]
475921  DNAH17; dynein heavy chain 17, axonemal isoform X2 [KO:K10408]
480114  DNAH14; dynein heavy chain 14, axonemal isoform X3 [KO:K10408]
482473  DNALI1; axonemal dynein light intermediate polypeptide 1 isoform X2 [KO:K10410]
474749  DNAI1; dynein intermediate chain 1, axonemal isoform X2 [KO:K10409]
483284  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
610370  DNAL1; dynein light chain 1, axonemal [KO:K10411]
474505  DNAL4; dynein light chain 4, axonemal isoform X2 [KO:K10412]
478918  TUBA4A; tubulin alpha-4A chain isoform X2 [KO:K07374]
486762  TUBA8; tubulin alpha-8 chain isoform X12 [KO:K07374]
608051  tubulin alpha-3 chain [KO:K07374]
477570  tubulin alpha-3 chain [KO:K07374]
487148  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
610636  tubulin alpha-1B chain isoform X2 [KO:K07374]
100856405  TUBA1C; tubulin alpha-1C chain [KO:K07374]
106557476  tubulin alpha-1A chain [KO:K07374]
100855995  tubulin alpha-8 chain-like isoform X3 [KO:K07374]
119866377  tubulin alpha-1B chain [KO:K07374]
474830  TUBB; tubulin beta chain [KO:K07375]
485950  TUBB1; tubulin beta-1 chain [KO:K07375]
480213  TUBB6; tubulin beta-6 chain [KO:K07375]
478702  tubulin beta-2B chain [KO:K07375]
491231  LOW QUALITY PROTEIN: tubulin beta chain [KO:K07375]
478701  TUBB2A; tubulin beta-2A chain [KO:K07375]
100855815  TUBB4A; tubulin beta-4A chain isoform X1 [KO:K07375]
102153202  TUBB4B; tubulin beta-4B chain isoform X2 [KO:K07375]
102154194  TUBB3; tubulin beta-3 chain [KO:K07375]
477746  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
478464  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
474652  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
474416  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
476144  KIF5C; kinesin heavy chain [KO:K10396]
100855651  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
474909  KLC4; kinesin light chain 4 [KO:K10407]
483715  KLC2; kinesin light chain 2 [KO:K10407]
484448  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
100855591  KLC1; kinesin light chain 1 isoform X10 [KO:K10407]
478553  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
488473  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
403567  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
485146  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
403928  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
477160  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
485773  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
476034  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X2 [KO:K05858] [EC:3.1.4.11]
100856190  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
100855965  STX1A; syntaxin-1A isoform X1 [KO:K04560]
480660  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
494007  GRIN1; glutamate receptor ionotropic, NMDA 1 precursor [KO:K05208]
494009  GRIN2B; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210]
476548  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
489462  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
489168  GRIA1; glutamate receptor 1 isoform X4 [KO:K05197]
482667  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
492110  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
489427  GRIA4; glutamate receptor 4 isoform X8 [KO:K05200]
485867  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
403748  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
403750  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
488635  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 isoform X1 [KO:K05003]
482163  REST; RE1-silencing transcription factor [KO:K09222]
100682666  RE1-silencing transcription factor-like [KO:K09222]
478372  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
612620  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
487309  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
475035  HDAC2; histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
479483  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
475152  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
478109  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
483863  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
611859  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
474512  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
476052  POLR2G; DNA-directed RNA polymerase II subunit RPB7 isoform X2 [KO:K03015]
478656  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
476476  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
111090847  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
100686835  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
100688583  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
119870926  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
403461  BDNF; brain-derived neurotrophic factor precursor [KO:K04355]
479866  CREBBP; CREB-binding protein isoform X8 [KO:K04498] [EC:2.3.1.48]
100684945  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
490507  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
477280  TAF4; transcription initiation factor TFIID subunit 4 isoform X5 [KO:K03129]
476209  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
611193  TBP; TATA-box-binding protein [KO:K03120]
611011  TBPL2; TATA box-binding protein-like 2 isoform X5 [KO:K03120]
486507  SP1; transcription factor Sp1 isoform X2 [KO:K04684]
607922  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
611934  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X2 [KO:K09048]
483636  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
491369  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
485046  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
612303  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
475266  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X5 [KO:K09047]
479127  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 [KO:K07202]
403606  PPARG; peroxisome proliferator-activated receptor gamma [KO:K08530]
488989  TFAM; transcription factor A, mitochondrial [KO:K11830]
482260  NRF1; nuclear respiratory factor 1 isoform X2 [KO:K11831]
403559  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
476258  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
442961  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
480356  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
475348  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
403877  GPX5; epididymal secretory glutathione peroxidase precursor [KO:K00432] [EC:1.11.1.9]
489179  GPX3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
488328  GPX6; glutathione peroxidase 6 precursor [KO:K00432] [EC:1.11.1.9]
478059  GPX8; probable glutathione peroxidase 8 [KO:K00432] [EC:1.11.1.9]
403574  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
403869  TP53; cellular tumor antigen p53 [KO:K04451]
403523  BAX; apoptosis regulator BAX [KO:K02159]
612153  BBC3; bcl-2-binding component 3 [KO:K10132]
804476  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
804477  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
804481  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
804483  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
804482  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
804484  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
804485  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
476004  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
480204  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2 [KO:K03943] [EC:7.1.1.2]
478421  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
102154035  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
481033  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
102154025  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
106557778  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
478032  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
100855914  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
102151506  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
607407  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X2 [KO:K03948]
100856334  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100682956  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100684768  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100686889  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100688724  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
119874008  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
475214  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100683951  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949]
100688835  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
119870103  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
474483  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
476722  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
480741  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
477718  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
608244  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10 isoform X1 [KO:K03954]
479805  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100683682  acyl carrier protein, mitochondrial-like [KO:K03955]
476735  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
475428  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
476659  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
111096466  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100683846  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100855975  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X2 [KO:K03959]
478577  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
478639  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
102154590  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial-like [KO:K03961]
100687033  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
476686  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
477798  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
475094  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
479887  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
480894  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
478880  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
478981  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
475978  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
479335  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X11 [KO:K03937]
475322  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
478629  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
476754  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X3 [KO:K03940] [EC:7.1.1.2]
610413  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
607943  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
476794  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
478634  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
478217  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
478983  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
476503  UQCRFS1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
804486  CYTB; cytochrome b [KO:K00412]
475121  CYC1; cytochrome c1, heme protein, mitochondrial [KO:K00413]
608455  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
479815  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
608530  UQCRH; cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
100683828  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 7-like [KO:K00417]
609744  UQCRQ; cytochrome b-c1 complex subunit 8 [KO:K00418]
486347  UQCR10; cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
611791  UQCR11; cytochrome b-c1 complex subunit 10 [KO:K00420]
804480  COX3; cytochrome c oxidase subunit III [KO:K02262]
804478  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
804479  COX2; cytochrome c oxidase subunit II [KO:K02261]
479623  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
485825  COX4I2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
478370  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
474567  COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
479780  COX6A2; cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
477508  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
102156968  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
612644  cytochrome c oxidase subunit 6B1 [KO:K02267]
100686580  cytochrome c oxidase subunit 6B1-like [KO:K02267]
102156642  cytochrome c oxidase subunit 6B1-like [KO:K02267]
119870901  cytochrome c oxidase subunit 6B2 [KO:K02267]
475739  COX7A2L; cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X3 [KO:K02270]
612614  COX7A1; cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
119863903  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
100686500  cytochrome c oxidase subunit 7A2, mitochondrial isoform X4 [KO:K02270]
100684983  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100687434  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100684842  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
102152785  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100686510  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100683076  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
609990  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
102155410  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
102151754  cytochrome c oxidase subunit 7C, mitochondrial-like isoform X5 [KO:K02272]
102153059  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
476040  COX8A; cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
106558390  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
480149  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
487689  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
403669  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
478009  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
476756  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
102153397  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
804488  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
491061  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
477595  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
478807  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100682717  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100685007  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
100685227  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
102154372  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
610725  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
102151342  ATP synthase subunit d, mitochondrial-like [KO:K02138]
482600  ATP synthase subunit d, mitochondrial-like isoform X2 [KO:K02138]
100684996  ATP synthase subunit d, mitochondrial [KO:K02138]
119870484  ATP synthase subunit d, mitochondrial [KO:K02138]
478410  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100682783  ATP synthase-coupling factor 6, mitochondrial-like [KO:K02131]
100685720  LOW QUALITY PROTEIN: ATP synthase-coupling factor 6, mitochondrial-like isoform X2 [KO:K02131]
804487  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
474681  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
102152873  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
479255  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
606963  VDAC3; voltage-dependent anion-selective channel protein 3 isoform 1 [KO:K15041]
475630  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
480830  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
492093  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
483832  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
610502  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
475258  CYCS; cytochrome c [KO:K08738]
100689004  LOW QUALITY PROTEIN: cytochrome c-like [KO:K08738]
482620  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
487432  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
480290  PSMA6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
100686569  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
100687060  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
475870  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100687556  LOW QUALITY PROTEIN: proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
119874448  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
404305  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100856395  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
100856348  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
476867  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
102152879  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
100683681  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
100686432  proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
610188  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
480338  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
607466  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100686423  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
480537  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
475338  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
100855659  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
475040  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
475848  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
475896  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100688636  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100855613  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
478522  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100687259  26S proteasome regulatory subunit 10B [KO:K03064]
475980  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
100687152  26S proteasome regulatory subunit 6A-like [KO:K03065]
480478  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
478654  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100683530  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
491018  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
477467  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
480465  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X2 [KO:K03035]
480610  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
484700  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X2 [KO:K03037]
479671  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
475934  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100192385  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
478765  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
476470  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100855986  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
608719  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
484504  MAP3K10; mitogen-activated protein kinase kinase kinase 10 isoform X1 [KO:K04418] [EC:2.7.11.25]
610766  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X6 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
480673  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
476215  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
485004  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
478232  MTOR; serine/threonine-protein kinase mTOR isoform X5 [KO:K07203] [EC:2.7.11.1]
477437  ULK1; serine/threonine-protein kinase ULK1 isoform X3 [KO:K21357] [EC:2.7.11.1]
489525  ULK2; serine/threonine-protein kinase ULK2 isoform X8 [KO:K08269] [EC:2.7.11.1]
486535  ATG101; autophagy-related protein 101 [KO:K19730]
483632  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
477876  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
483634  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
480513  BECN1; beclin-1 isoform X1 [KO:K08334]
490371  BECN2; beclin-2 isoform X1 [KO:K08334]
477064  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
490704  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
479220  NRBF2; nuclear receptor-binding factor 2 isoform X2 [KO:K21246]
490477  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
480428  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
483756  ATG2A; autophagy-related protein 2 homolog A isoform X2 [KO:K17906]
490899  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X2 [KO:K17908]
608721  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X8 [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
cfa00190  Oxidative phosphorylation
cfa03020  RNA polymerase
cfa03022  Basal transcription factors
cfa03050  Proteasome
cfa04020  Calcium signaling pathway
cfa04115  p53 signaling pathway
cfa04140  Autophagy - animal
cfa04141  Protein processing in endoplasmic reticulum
cfa04144  Endocytosis
cfa04210  Apoptosis
cfa04724  Glutamatergic synapse
KO pathway
ko05016   

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Genome (1)   
   KEGG GENOME (1)   
Gene (350)   
   KEGG GENES (350)   
All databases (351)   

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