KEGG   PATHWAY: panu05016
Entry
panu05016                   Pathway                                
Name
Huntington disease - Papio anubis (olive baboon)
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
panu05016  Huntington disease
panu05016

Organism
Papio anubis (olive baboon) [GN:panu]
Gene
101010676  CLTA; clathrin light chain A isoform X1 [KO:K04644]
101015474  CLTB; LOW QUALITY PROTEIN: clathrin light chain B [KO:K04645]
101017523  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
101022293  CLTC; clathrin heavy chain 1 isoform X2 [KO:K04646]
101006933  HIP1; huntingtin-interacting protein 1 isoform X2 [KO:K04559]
101016499  AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
101016056  AP2A1; AP-2 complex subunit alpha-1 isoform X2 [KO:K11824]
101020304  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
101008695  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
101014753  AP2S1; AP-2 complex subunit sigma isoform X2 [KO:K11827]
101000758  HTT; huntingtin [KO:K04533]
101016241  DCTN1; dynactin subunit 1 isoform X6 [KO:K04648]
100999496  DCTN2; dynactin subunit 2 isoform X3 [KO:K10424]
101006034  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
101017720  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
101015822  DCTN5; dynactin subunit 5 isoform X2 [KO:K10427]
101008288  DCTN6; dynactin subunit 6 [KO:K10428]
101007489  ACTR1B; beta-centractin isoform X1 [KO:K16575]
101023419  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
101016438  ACTR10; actin-related protein 10 isoform X1 [KO:K16576]
101021843  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
101013747  DNAH7; dynein heavy chain 7, axonemal isoform X1 [KO:K10408]
103876134  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
101017895  dynein heavy chain 14, axonemal [KO:K10408]
101013562  dynein heavy chain 17, axonemal [KO:K10408]
101014725  DNAH9; dynein heavy chain 9, axonemal isoform X1 [KO:K10408]
101016123  DNAH6; dynein heavy chain 6, axonemal isoform X1 [KO:K10408]
101023096  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
101026626  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
101013413  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
101019979  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
101013563  DNAH8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
101009834  DNAH2; dynein heavy chain 2, axonemal isoform X3 [KO:K10408]
101002935  DNAH3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
101016231  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
101007991  DNAI1; dynein intermediate chain 1, axonemal isoform X2 [KO:K10409]
101005389  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
101006108  DNAL4; dynein light chain 4, axonemal [KO:K10412]
101010758  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
101018365  tubulin alpha-1C chain isoform X1 [KO:K07374]
101006121  tubulin alpha-4A chain isoform X2 [KO:K07374]
101001204  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
101017171  LOW QUALITY PROTEIN: tubulin alpha chain [KO:K07374]
101016030  LOW QUALITY PROTEIN: tubulin beta-2A chain [KO:K07375]
101003610  TUBB4A; tubulin beta-4A chain [KO:K07375]
101016760  tubulin beta-2B chain isoform X1 [KO:K07375]
100998701  TUBB; tubulin beta chain [KO:K07375]
101005103  TUBB4B; tubulin beta-4B chain [KO:K07375]
100999710  tubulin beta-8 chain-like [KO:K07375]
116273103  tubulin beta-8 chain-like [KO:K07375]
101018350  tubulin beta-8 chain [KO:K07375]
100137440  TUBB2A; tubulin beta-2A chain [KO:K07375]
101005758  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
101014512  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
110742076  TUBB3; tubulin beta-3 chain [KO:K07375]
101005010  MAPK10; mitogen-activated protein kinase 10 isoform X3 [KO:K04440] [EC:2.7.11.24]
101022320  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
101019884  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
101002273  KIF5A; kinesin heavy chain [KO:K10396]
101014566  KIF5C; kinesin heavy chain [KO:K10396]
101002261  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
101026784  KLC3; kinesin light chain 3 isoform X3 [KO:K10407]
101000901  KLC2; kinesin light chain 2 [KO:K10407]
101010092  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
101022226  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
101014946  IFT57; intraflagellar transport protein 57 homolog isoform X2 [KO:K04638]
101009470  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
101023728  CASP3; caspase-3 isoform X2 [KO:K02187] [EC:3.4.22.56]
101020156  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
101024177  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X2 [KO:K04634]
101012903  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
100998763  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
100999120  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X5 [KO:K05858] [EC:3.1.4.11]
101010000  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X4 [KO:K05858] [EC:3.1.4.11]
101012925  STX1A; syntaxin-1A [KO:K04560]
101001959  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X3 [KO:K05208]
101009230  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101017317  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
101018290  DLG4; disks large homolog 4 isoform X5 [KO:K11828]
101000382  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
101026424  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
101020351  GRIA3; glutamate receptor 3 isoform X2 [KO:K05199]
101012565  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
101020604  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
101001168  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
101007382  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
101022028  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
101007427  REST; RE1-silencing transcription factor isoform X1 [KO:K09222]
101016208  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
101015132  RCOR1; REST corepressor 1 isoform X1 [KO:K11829]
101024777  HDAC2; histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
101010160  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
100998821  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
101005833  POLR2B; DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
101006304  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
101011026  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
110741273  POLR2E; LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
101026595  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
101006252  POLR2G; DNA-directed RNA polymerase II subunit RPB7 isoform X2 [KO:K03015]
101002187  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
101021248  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
110743057  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
110741160  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
100997880  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
108585500  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
101007866  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
101027061  EP300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
101002586  CREBBP; CREB-binding protein isoform X5 [KO:K04498] [EC:2.3.1.48]
101000177  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
101015450  TAF4B; transcription initiation factor TFIID subunit 4B isoform X2 [KO:K03129]
101018561  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
101006949  TBP; TATA-box-binding protein [KO:K03120]
101003400  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
116268873  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
101017891  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
101004296  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
101020406  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
101004434  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
101008452  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
101007514  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X4 [KO:K09047]
101006519  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
101012073  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
116268550  transcription factor A, mitochondrial [KO:K11830]
101002613  TFAM; transcription factor A, mitochondrial [KO:K11830]
101015737  transcription factor A, mitochondrial [KO:K11830]
101002669  NRF1; nuclear respiratory factor 1 isoform X2 [KO:K11831]
101009607  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
101000707  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
101009135  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
101023235  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
101018549  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
101018890  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
101020922  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
101017505  GPX2; glutathione peroxidase 2 isoform X1 [KO:K00432] [EC:1.11.1.9]
103885101  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
101003615  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
101006608  TP53; cellular tumor antigen p53 [KO:K04451]
101021738  BBC3; bcl-2-binding component 3 isoform X1 [KO:K10132]
14444642  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
14444630  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
14444636  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
14444638  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
14444637  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
14444639  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
116273132  LOW QUALITY PROTEIN: uncharacterized protein LOC116273132 [KO:K03884] [EC:7.1.1.2]
14444640  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
101006922  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100998941  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
101009040  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
101007924  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
101013937  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
103885307  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
101025484  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
108580506  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101014495  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
108580495  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101026476  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
108586421  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101021591  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
100999652  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101013121  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
101008045  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951]
101025936  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
101008424  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
101006233  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X3 [KO:K03954]
103876537  acyl carrier protein, mitochondrial [KO:K03955]
101024176  acyl carrier protein, mitochondrial-like [KO:K03955]
116272890  acyl carrier protein, mitochondrial [KO:K03955]
101007204  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
101026186  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
101002746  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
101001064  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
101019309  NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
101021838  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958]
101005887  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
108587044  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
101004754  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
101026496  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
101002541  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
101015506  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6-like [KO:K03962]
100997770  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
101026190  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
101003419  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
101022237  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
101022093  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
101014764  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
101001230  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
101002291  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
101020754  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
101017486  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
116275293  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
101026904  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
101002763  NDUFS8; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100998717  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101017439  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
101025145  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
116273554  succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
101000070  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101001260  succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
101006239  succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
101002161  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
101011036  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
110741279  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
101026782  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
14444641  CYTB; cytochrome b [KO:K00412]
101021397  cytochrome c1, heme protein, mitochondrial [KO:K00413]
101009600  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
101026315  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
101006001  cytochrome b-c1 complex subunit 6, mitochondrial isoform X2 [KO:K00416]
100997829  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
101017841  cytochrome b-c1 complex subunit 8 [KO:K00418]
100997845  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
116273763  cytochrome b-c1 complex subunit 10 [KO:K00420]
14444635  COX3; cytochrome c oxidase subunit III [KO:K02262]
14444631  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
14444632  COX2; cytochrome c oxidase subunit II [KO:K02261]
101006909  cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
101019045  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
101003461  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
101020469  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
101001315  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
101006649  COX5A; cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
103877721  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
101007148  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
103876119  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
101000271  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
101018921  cytochrome c oxidase subunit 6B2 [KO:K02267]
101010708  cytochrome c oxidase subunit 6B1 [KO:K02267]
108586074  cytochrome c oxidase subunit 6C-like [KO:K02268]
101013819  cytochrome c oxidase subunit 6C [KO:K02268]
101002878  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
101019499  cytochrome c oxidase subunit 7A2, mitochondrial isoform X1 [KO:K02270]
116271663  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
116271667  cytochrome c oxidase subunit 7A1, mitochondrial isoform X1 [KO:K02270]
101021606  cytochrome c oxidase subunit 7A1, mitochondrial isoform X2 [KO:K02270]
101013709  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
116269782  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
103884346  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
108581070  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108585455  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
101014053  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
108586679  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108587428  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108586288  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116274896  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116274898  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
108585269  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
101019295  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
101026809  cytochrome c oxidase subunit 8C, mitochondrial isoform X2 [KO:K02273]
100126727  COX8A; cytochrome c oxidase subunit 8A, mitochondrial precursor [KO:K02273]
100997548  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
101017406  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
101010257  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100137597  ATP5C1; ATP synthase subunit gamma, mitochondrial [KO:K02136]
101005176  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
14444634  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
101000308  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial isoform X2 [KO:K02127]
101007596  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
101021172  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
101011935  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
101017933  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
101018964  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
101024769  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
101007673  ATP synthase subunit d, mitochondrial [KO:K02138]
101016617  ATP synthase subunit O, mitochondrial [KO:K02137]
100137250  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
101005105  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
14444633  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
101020566  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
101009552  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
101019427  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
100998956  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
101009750  SLC25A31; ADP/ATP translocase 4 isoform X1 [KO:K05863]
101025041  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
101000631  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100997257  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
101025906  cytochrome c-like [KO:K08738]
101020480  cytochrome c-like [KO:K08738]
101021327  cytochrome c-like [KO:K08738]
101014039  cytochrome c-like [KO:K08738]
101008579  cytochrome c [KO:K08738]
100997529  cytochrome c [KO:K08738]
101021349  cytochrome c-like [KO:K08738]
100997503  cytochrome c-like [KO:K08738]
101019410  cytochrome c-like [KO:K08738]
103878393  cytochrome c-like [KO:K08738]
108582823  cytochrome c-like [KO:K08738]
101014473  cytochrome c-like [KO:K08738]
101023208  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
101009907  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
101022470  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
101021214  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
101000967  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
101014380  PSMA8; proteasome subunit alpha-type 8 isoform X2 [KO:K02731] [EC:3.4.25.1]
100999118  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
101007366  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
101020989  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
101013344  PSMA3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
116275734  proteasome subunit alpha type-3-like [KO:K02727] [EC:3.4.25.1]
101018540  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
101009488  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101024402  PSMB3; proteasome subunit beta type-3 isoform X1 [KO:K02735] [EC:3.4.25.1]
101002516  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100997814  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
101007533  PSMB1; proteasome subunit beta type-1 isoform X2 [KO:K02732] [EC:3.4.25.1]
116272469  proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100137420  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
101005223  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
101006552  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
101016782  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
101018096  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100998789  26S proteasome regulatory subunit 10B [KO:K03064]
101022271  PSMC3; 26S proteasome regulatory subunit 6A isoform X2 [KO:K03065]
101002087  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
101005799  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
101016848  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
101013675  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
101025669  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
101018302  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
101002325  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100997045  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
101014998  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 isoform X3 [KO:K03038]
101026980  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
100137416  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
101021787  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
101022085  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
101024714  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X2 [KO:K06691]
101027004  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
101023378  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
101001514  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101017914  TRAF2; LOW QUALITY PROTEIN: TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
101002917  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
101011807  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
101015041  MTOR; serine/threonine-protein kinase mTOR isoform X2 [KO:K07203] [EC:2.7.11.1]
101010161  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
101005224  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
101022496  ATG101; autophagy-related protein 101 [KO:K19730]
101005094  ATG13; autophagy-related protein 13 isoform X8 [KO:K08331]
101009883  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
101004741  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X2 [KO:K17985]
100999381  beclin-2 [KO:K08334]
101012227  BECN1; beclin-1 isoform X5 [KO:K08334]
116273742  BECN2; beclin-2 [KO:K08334]
116273743  beclin-2 [KO:K08334]
101011963  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
101001656  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
101017200  nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101019324  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101022653  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
101015836  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
100137474  ATG2A; autophagy-related protein 2 homolog A [KO:K17906]
101000917  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X5 [KO:K17908]
101016439  WD repeat domain phosphoinositide-interacting protein 2-like isoform X2 [KO:K17908]
101024767  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
Compound
C00025  L-Glutamate
C00076  Calcium cation
C00238  Potassium cation
C01245  D-myo-Inositol 1,4,5-trisphosphate
C01330  Sodium cation
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
Reference
PMID:15459747
  Authors
Landles C, Bates GP
  Title
Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series.
  Journal
EMBO Rep 5:958-63 (2004)
DOI:10.1038/sj.embor.7400250
Reference
PMID:17041811
  Authors
Borrell-Pages M, Zala D, Humbert S, Saudou F
  Title
Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.
  Journal
Cell Mol Life Sci 63:2642-60 (2006)
DOI:10.1007/s00018-006-6242-0
Reference
PMID:18951640
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
DOI:10.1016/j.tins.2008.09.004
Reference
PMID:16288298
  Authors
Cattaneo E, Zuccato C, Tartari M
  Title
Normal huntingtin function: an alternative approach to Huntington's disease.
  Journal
Nat Rev Neurosci 6:919-30 (2005)
DOI:10.1038/nrn1806
Reference
PMID:15336977
  Authors
Bezprozvanny I, Hayden MR
  Title
Deranged neuronal calcium signaling and Huntington disease.
  Journal
Biochem Biophys Res Commun 322:1310-7 (2004)
DOI:10.1016/j.bbrc.2004.08.035
Reference
PMID:14526190
  Authors
Sawa A, Tomoda T, Bae BI
  Title
Mechanisms of neuronal cell death in Huntington's disease.
  Journal
Cytogenet Genome Res 100:287-95 (2003)
DOI:10.1159/000072864
Reference
PMID:12372277
  Authors
Ross CA.
  Title
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
  Journal
Neuron 35:819-22 (2002)
DOI:10.1016/S0896-6273(02)00872-3
Reference
PMID:15242639
  Authors
Ross CA
  Title
Huntington's disease: new paths to pathogenesis.
  Journal
Cell 118:4-7 (2004)
DOI:10.1016/j.cell.2004.06.022
Reference
PMID:24739785
  Authors
Coffey ET
  Title
Nuclear and cytosolic JNK signalling in neurons.
  Journal
Nat Rev Neurosci 15:285-99 (2014)
DOI:10.1038/nrn3729
Reference
PMID:19525941
  Authors
Morfini GA, You YM, Pollema SL, Kaminska A, Liu K, Yoshioka K, Bjorkblom B, Coffey ET, Bagnato C, Han D, Huang CF, Banker G, Pigino G, Brady ST
  Title
Pathogenic huntingtin inhibits fast axonal transport by activating JNK3 and phosphorylating kinesin.
  Journal
Nat Neurosci 12:864-71 (2009)
DOI:10.1038/nn.2346
Reference
PMID:26920688
  Authors
Kumar P, Kumar D, Jha SK, Jha NK, Ambasta RK
  Title
Ion Channels in Neurological Disorders.
  Journal
Adv Protein Chem Struct Biol 103:97-136 (2016)
DOI:10.1016/bs.apcsb.2015.10.006
Reference
PMID:25191533
  Authors
Proft J, Weiss N
  Title
Rectifying rectifier channels in Huntington disease.
  Journal
Commun Integr Biol 7:e29410 (2014)
DOI:10.4161/cib.29410
Reference
PMID:15695335
  Authors
Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I
  Title
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
  Journal
Proc Natl Acad Sci U S A 102:2602-7 (2005)
DOI:10.1073/pnas.0409402102
Reference
PMID:29427371
  Authors
Mackay JP, Nassrallah WB, Raymond LA
  Title
Cause or compensation?-Altered neuronal Ca(2+) handling in Huntington's disease.
  Journal
CNS Neurosci Ther 24:301-310 (2018)
DOI:10.1111/cns.12817
Reference
PMID:11807533
  Authors
Mattson MP.
  Title
Accomplices to neuronal death.
  Journal
Nature 415:377-9 (2002)
DOI:10.1038/415377a
Reference
PMID:17081970
  Authors
McGill JK, Beal MF
  Title
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Journal
Cell 127:465-8 (2006)
DOI:10.1016/j.cell.2006.10.023
Reference
PMID:30941017
  Authors
Intihar TA, Martinez EA, Gomez-Pastor R
  Title
Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1alpha Transcription Factors.
  Journal
Front Cell Neurosci 13:103 (2019)
DOI:10.3389/fncel.2019.00103
Reference
PMID:15996541
  Authors
La Spada AR, Morrison RS
  Title
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
  Journal
Neuron 47:1-3 (2005)
DOI:10.1016/j.neuron.2005.06.023
Reference
PMID:11738471
  Authors
Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
  Journal
Neurochem Int 40:37-52 (2002)
DOI:10.1016/S0197-0186(01)00059-6
Reference
PMID:15996546
  Authors
Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A
  Title
p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
  Journal
Neuron 47:29-41 (2005)
DOI:10.1016/j.neuron.2005.06.005
Reference
PMID:11839795
  Authors
Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ
  Title
Interaction of Huntington disease protein with transcriptional activator Sp1.
  Journal
Mol Cell Biol 22:1277-87 (2002)
DOI:10.1128/MCB.22.5.1277-1287.2002
Reference
PMID:25270767
  Authors
Vidal RL, Matus S, Bargsted L, Hetz C
  Title
Targeting autophagy in neurodegenerative diseases.
  Journal
Trends Pharmacol Sci 35:583-91 (2014)
DOI:10.1016/j.tips.2014.09.002
Reference
PMID:16522639
  Authors
Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J
  Title
Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1.
  Journal
J Biol Chem 281:14474-85 (2006)
DOI:10.1074/jbc.M600364200
Related
pathway
panu00190  Oxidative phosphorylation
panu03020  RNA polymerase
panu03022  Basal transcription factors
panu03050  Proteasome
panu04020  Calcium signaling pathway
panu04115  p53 signaling pathway
panu04140  Autophagy - animal
panu04141  Protein processing in endoplasmic reticulum
panu04144  Endocytosis
panu04210  Apoptosis
panu04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

DBGET integrated database retrieval system