KEGG   PATHWAY: ecb05016
Entry
ecb05016                    Pathway                                
Name
Huntington disease - Equus caballus (horse)
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
ecb05016  Huntington disease
ecb05016

Organism
Equus caballus (horse) [GN:ecb]
Gene
100055375  CLTA; clathrin light chain A isoform X1 [KO:K04644]
100068882  CLTB; clathrin light chain B isoform X3 [KO:K04645]
100057364  CLTC; clathrin heavy chain 1 isoform X2 [KO:K04646]
100053137  clathrin heavy chain 2 isoform X1 [KO:K04646]
100061280  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
100056103  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
100056318  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
100058050  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
100058961  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
100071193  AP2S1; AP-2 complex subunit sigma [KO:K11827]
100053721  HTT; huntingtin isoform X1 [KO:K04533]
100053938  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
100050464  DCTN2; dynactin subunit 2 isoform X3 [KO:K10424]
100068546  DCTN3; dynactin subunit 3 isoform X2 [KO:K10425]
100071625  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
100068373  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
100058409  DCTN6; dynactin subunit 6 [KO:K10428]
100069908  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
100051108  ACTR1B; beta-centractin isoform X1 [KO:K16575]
100058966  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
100066559  HAP1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
100060282  DNAH1; dynein heavy chain 1, axonemal isoform X2 [KO:K10408]
100146115  DNAH2; dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
100059138  DNAH3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
100070297  DNAH7; dynein heavy chain 7, axonemal isoform X1 [KO:K10408]
100070308  DNAH5; dynein heavy chain 5, axonemal isoform X1 [KO:K10408]
100067558  DNAH6; dynein heavy chain 6, axonemal isoform X1 [KO:K10408]
100073091  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
100050892  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
100065248  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
100061177  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
100057640  DNAH17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
100056953  DNAH14; dynein heavy chain 14, axonemal isoform X1 [KO:K10408]
102150627  DNAH11; dynein heavy chain 11, axonemal isoform X1 [KO:K10408]
100069142  DNALI1; LOW QUALITY PROTEIN: axonemal dynein light intermediate polypeptide 1 [KO:K10410]
100068649  DNAI1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409]
100060918  DNAI2; dynein intermediate chain 2, axonemal isoform X1 [KO:K11143]
100050368  DNAL1; dynein light chain 1, axonemal [KO:K10411]
100070185  DNAL4; dynein light chain 4, axonemal [KO:K10412]
100059285  tubulin alpha-1D chain [KO:K07374]
100051840  TUBA1A; tubulin alpha-1A chain [KO:K07374]
100051901  tubulin alpha-1C chain [KO:K07374]
100052388  tubulin alpha-3 chain [KO:K07374]
100054576  TUBA8; tubulin alpha-8 chain isoform X2 [KO:K07374]
100065732  tubulin alpha-3 chain [KO:K07374]
100059249  TUBA4A; tubulin alpha-4A chain [KO:K07374]
100146855  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
100059091  tubulin alpha-1B chain [KO:K07374]
100072403  tubulin alpha-3 chain [KO:K07374]
100050361  tubulin beta-2B chain [KO:K07375]
100056684  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
100051013  TUBB; tubulin beta chain [KO:K07375]
100058421  TUBB6; tubulin beta-6 chain [KO:K07375]
100629875  TUBB2A; tubulin beta-2A chain [KO:K07375]
100059595  TUBB4B; tubulin beta-4B chain [KO:K07375]
100065776  TUBB4A; tubulin beta-4A chain [KO:K07375]
111772826  TUBB3; tubulin beta-3 chain [KO:K07375]
100052608  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
100057961  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100051798  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100061228  KIF5B; kinesin-1 heavy chain [KO:K10396]
100050542  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
100056413  KIF5C; kinesin heavy chain isoform X1 [KO:K10396]
100055017  KLC4; kinesin light chain 4 [KO:K10407]
100055290  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
100147291  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
100052297  KLC2; kinesin light chain 2 [KO:K10407]
100061752  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
100066502  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
100034083  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
100060364  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
100063136  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634]
100057315  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
100051558  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
100055407  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
100051485  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
100061863  STX1A; syntaxin-1A [KO:K04560]
100058760  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
100059861  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
100063746  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100052808  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
100061544  DLG4; disks large homolog 4 isoform X8 [KO:K11828]
100060020  GRIA1; glutamate receptor 1 isoform X4 [KO:K05197]
100069331  GRIA2; glutamate receptor 2 isoform 1 precursor [KO:K05198]
100055136  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
100061561  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
100070046  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
100053647  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
100067133  SLC1A2; excitatory amino acid transporter 2 isoform X4 [KO:K05613]
100058121  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
100058152  REST; RE1-silencing transcription factor [KO:K09222]
100051174  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
100058618  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
100072578  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
100070281  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
100072997  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
100053708  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
100052195  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
100069214  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
100065431  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
100054935  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
100063883  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
100059115  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
100051926  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
100629865  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
111776015  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
100059695  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
100630612  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
100009689  BDNF; brain-derived neurotrophic factor precursor [KO:K04355]
100055626  EP300; histone acetyltransferase p300 isoform X2 [KO:K04498] [EC:2.3.1.48]
100069613  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
100057726  TAF4; LOW QUALITY PROTEIN: transcription initiation factor TFIID subunit 4 [KO:K03129]
100064447  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
100067729  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
100049846  TBP; TATA-box-binding protein [KO:K03120]
100629527  SP1; transcription factor Sp1 [KO:K04684]
100066388  CREB1; cyclic AMP-responsive element-binding protein 1 [KO:K05870]
100062307  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
100064828  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
100057002  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
100067794  CREB3; LOW QUALITY PROTEIN: cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100063153  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
100054803  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
100055716  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 [KO:K07202]
100051258  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
100062631  TFAM; transcription factor A, mitochondrial isoform X2 [KO:K11830]
100056975  NRF1; nuclear respiratory factor 1 isoform X2 [KO:K11831]
100033855  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100034223  SOD2; superoxide dismutase [Mn], mitochondrial precursor [KO:K04564] [EC:1.15.1.1]
100033835  GPX3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
100034079  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
100050590  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
100146145  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100053396  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
100061078  GPX6; glutathione peroxidase 6 precursor [KO:K00432] [EC:1.11.1.9]
100052064  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
102150050  glutathione peroxidase 1-like [KO:K00432] [EC:1.11.1.9]
100063105  UCP1; LOW QUALITY PROTEIN: mitochondrial brown fat uncoupling protein 1 [KO:K08769]
100062044  TP53; cellular tumor antigen p53 [KO:K04451]
100054674  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
100146714  BBC3; bcl-2-binding component 3 [KO:K10132]
807846  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
807844  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
807855  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
807852  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
807851  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
807853  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
807848  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100059569  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100054445  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
100057691  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X4 [KO:K03944]
100058835  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100061811  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
111772123  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
111767785  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
111767790  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
100052169  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
100052985  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
106783042  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100071532  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
100066991  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100067562  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100051504  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100057698  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
100069079  NDUFAB1; acyl carrier protein, mitochondrial isoform X1 [KO:K03955]
100065070  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
100146253  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100630425  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
100064695  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
100067936  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100070815  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100058402  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
100630340  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
100064992  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 isoform X2 [KO:K03963]
100060451  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
100057623  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100065553  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100060719  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
100066330  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X2 [KO:K03934] [EC:7.1.1.2]
100066089  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
100058223  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936] [EC:7.1.1.2]
100063168  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
100054377  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
100071879  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
100068855  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
100053392  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100629515  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100630892  NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100034244  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
100050091  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
100065940  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X3 [KO:K00236]
100629334  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
100053634  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
807847  CYTB; cytochrome b [KO:K00412]
100146668  CYC1; cytochrome c1, heme protein, mitochondrial [KO:K00413]
100053786  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial isoform X2 [KO:K00414]
100057847  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100051878  cytochrome b-c1 complex subunit 6, mitochondrial isoform X2 [KO:K00416]
100059627  UQCRB; cytochrome b-c1 complex subunit 7 [KO:K00417]
111767815  cytochrome b-c1 complex subunit 8 [KO:K00418]
100630846  cytochrome b-c1 complex subunit 9 [KO:K00419]
100147519  cytochrome b-c1 complex subunit 10 [KO:K00420]
807856  COX3; cytochrome c oxidase subunit III [KO:K02262]
807850  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
807845  COX2; cytochrome c oxidase subunit II [KO:K02261]
100053548  COX4I2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
100056171  COX4I1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
100051391  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
100061973  COX5B; cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
100053420  COX6A1; cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100063197  COX6A2; cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
100051249  COX6B1; cytochrome c oxidase subunit 6B1 [KO:K02267]
102149678  cytochrome c oxidase subunit 6B2 [KO:K02267]
100055813  cytochrome c oxidase subunit 6C [KO:K02268]
100053736  COX7A2L; cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100630209  LOW QUALITY PROTEIN: uncharacterized protein LOC100630209 [KO:K02270]
106782100  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
100630758  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
100629332  COX7B2; cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
100630549  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
111770393  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
111775959  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
100053178  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100059825  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
100069978  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X3 [KO:K02136]
100146850  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100630568  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
807854  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100058893  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100069523  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100053347  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100064131  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
100052170  ATP5PD; ATP synthase subunit d, mitochondrial isoform X2 [KO:K02138]
100052131  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100066379  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
807849  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100062974  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100064276  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
100050036  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
100050762  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100059103  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100071908  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100146128  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
100073016  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
100053958  CYCS; cytochrome c [KO:K08738]
111768755  cytochrome c, testis-specific [KO:K08738]
100052552  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
100054331  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
100050805  PSMA6; proteasome subunit alpha type-6 isoform X2 [KO:K02730] [EC:3.4.25.1]
100064293  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100050569  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
100064417  PSMA8; proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
100057828  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100061457  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
100056224  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
100050987  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
100072870  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
100067317  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100068477  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100055325  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100052570  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
100051957  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100059821  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100058456  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100062573  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
100064604  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100049903  PSMC6; 26S proteasome regulatory subunit 10B [KO:K03064]
100051468  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
100053987  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
100059081  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100057015  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X2 [KO:K03032]
100147612  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100058668  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
100063689  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100071770  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100055920  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100067363  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100050948  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
100216429  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
100051409  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X2 [KO:K03030]
100064192  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
102150324  SEM1; LOW QUALITY PROTEIN: 26S proteasome complex subunit SEM1 [KO:K10881]
100064339  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100068006  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
100073186  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
100147230  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
100051341  MTOR; serine/threonine-protein kinase mTOR isoform X3 [KO:K07203] [EC:2.7.11.1]
100073154  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
100062689  ATG101; autophagy-related protein 101 isoform X2 [KO:K19730]
100050734  ATG13; autophagy-related protein 13 [KO:K08331]
100050327  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
100050652  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X5 [KO:K17985]
100060790  BECN2; beclin-2 [KO:K08334]
100052295  BECN1; beclin-1 isoform X1 [KO:K08334]
100052647  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X2 [KO:K08333] [EC:2.7.11.1]
100050443  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
100072436  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
100053039  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
100050653  ATG2A; autophagy-related protein 2 homolog A isoform X4 [KO:K17906]
100054652  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
100053194  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X2 [KO:K17908]
100060823  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
Compound
C00025  L-Glutamate
C00076  Calcium cation
C00238  Potassium cation
C01245  D-myo-Inositol 1,4,5-trisphosphate
C01330  Sodium cation
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
Reference
PMID:15459747
  Authors
Landles C, Bates GP
  Title
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series.
  Journal
EMBO Rep 5:958-63 (2004)
DOI:10.1038/sj.embor.7400250
Reference
PMID:17041811
  Authors
Borrell-Pages M, Zala D, Humbert S, Saudou F
  Title
Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.
  Journal
Cell Mol Life Sci 63:2642-60 (2006)
DOI:10.1007/s00018-006-6242-0
Reference
PMID:18951640
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
DOI:10.1016/j.tins.2008.09.004
Reference
PMID:16288298
  Authors
Cattaneo E, Zuccato C, Tartari M
  Title
Normal huntingtin function: an alternative approach to Huntington's disease.
  Journal
Nat Rev Neurosci 6:919-30 (2005)
DOI:10.1038/nrn1806
Reference
PMID:15336977
  Authors
Bezprozvanny I, Hayden MR
  Title
Deranged neuronal calcium signaling and Huntington disease.
  Journal
Biochem Biophys Res Commun 322:1310-7 (2004)
DOI:10.1016/j.bbrc.2004.08.035
Reference
PMID:14526190
  Authors
Sawa A, Tomoda T, Bae BI
  Title
Mechanisms of neuronal cell death in Huntington's disease.
  Journal
Cytogenet Genome Res 100:287-95 (2003)
DOI:10.1159/000072864
Reference
PMID:12372277
  Authors
Ross CA.
  Title
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
  Journal
Neuron 35:819-22 (2002)
DOI:10.1016/S0896-6273(02)00872-3
Reference
PMID:15242639
  Authors
Ross CA
  Title
Huntington's disease: new paths to pathogenesis.
  Journal
Cell 118:4-7 (2004)
DOI:10.1016/j.cell.2004.06.022
Reference
PMID:24739785
  Authors
Coffey ET
  Title
Nuclear and cytosolic JNK signalling in neurons.
  Journal
Nat Rev Neurosci 15:285-99 (2014)
DOI:10.1038/nrn3729
Reference
PMID:19525941
  Authors
Morfini GA, You YM, Pollema SL, Kaminska A, Liu K, Yoshioka K, Bjorkblom B, Coffey ET, Bagnato C, Han D, Huang CF, Banker G, Pigino G, Brady ST
  Title
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin.
  Journal
Nat Neurosci 12:864-71 (2009)
DOI:10.1038/nn.2346
Reference
PMID:26920688
  Authors
Kumar P, Kumar D, Jha SK, Jha NK, Ambasta RK
  Title
Ion Channels in Neurological Disorders.
  Journal
Adv Protein Chem Struct Biol 103:97-136 (2016)
DOI:10.1016/bs.apcsb.2015.10.006
Reference
PMID:25191533
  Authors
Proft J, Weiss N
  Title
Rectifying rectifier channels in Huntington disease.
  Journal
Commun Integr Biol 7:e29410 (2014)
DOI:10.4161/cib.29410
Reference
PMID:15695335
  Authors
Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I
  Title
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
  Journal
Proc Natl Acad Sci U S A 102:2602-7 (2005)
DOI:10.1073/pnas.0409402102
Reference
PMID:29427371
  Authors
Mackay JP, Nassrallah WB, Raymond LA
  Title
Cause or compensation?-Altered neuronal Ca(2+) handling in Huntington's disease.
  Journal
CNS Neurosci Ther 24:301-310 (2018)
DOI:10.1111/cns.12817
Reference
PMID:11807533
  Authors
Mattson MP.
  Title
Accomplices to neuronal death.
  Journal
Nature 415:377-9 (2002)
DOI:10.1038/415377a
Reference
PMID:17081970
  Authors
McGill JK, Beal MF
  Title
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Journal
Cell 127:465-8 (2006)
DOI:10.1016/j.cell.2006.10.023
Reference
PMID:30941017
  Authors
Intihar TA, Martinez EA, Gomez-Pastor R
  Title
Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1alpha Transcription Factors.
  Journal
Front Cell Neurosci 13:103 (2019)
DOI:10.3389/fncel.2019.00103
Reference
PMID:15996541
  Authors
La Spada AR, Morrison RS
  Title
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
  Journal
Neuron 47:1-3 (2005)
DOI:10.1016/j.neuron.2005.06.023
Reference
PMID:11738471
  Authors
Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
  Journal
Neurochem Int 40:37-52 (2002)
DOI:10.1016/S0197-0186(01)00059-6
Reference
PMID:15996546
  Authors
Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A
  Title
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
  Journal
Neuron 47:29-41 (2005)
DOI:10.1016/j.neuron.2005.06.005
Reference
PMID:11839795
  Authors
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ
  Title
Interaction of Huntington disease protein with transcriptional activator Sp1.
  Journal
Mol Cell Biol 22:1277-87 (2002)
DOI:10.1128/MCB.22.5.1277-1287.2002
Reference
PMID:25270767
  Authors
Vidal RL, Matus S, Bargsted L, Hetz C
  Title
Targeting autophagy in neurodegenerative diseases.
  Journal
Trends Pharmacol Sci 35:583-91 (2014)
DOI:10.1016/j.tips.2014.09.002
Reference
PMID:16522639
  Authors
Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J
  Title
Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1.
  Journal
J Biol Chem 281:14474-85 (2006)
DOI:10.1074/jbc.M600364200
Related
pathway
ecb00190  Oxidative phosphorylation
ecb03020  RNA polymerase
ecb03022  Basal transcription factors
ecb03050  Proteasome
ecb04020  Calcium signaling pathway
ecb04115  p53 signaling pathway
ecb04140  Autophagy - animal
ecb04141  Protein processing in endoplasmic reticulum
ecb04144  Endocytosis
ecb04210  Apoptosis
ecb04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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