KEGG   PATHWAY: morg05016
Entry
morg05016                   Pathway                                
Name
Huntington disease - Microtus oregoni (creeping vole)
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
morg05016  Huntington disease
morg05016

Organism
Microtus oregoni (creeping vole) [GN:morg]
Gene
121458420  Clta; clathrin light chain A isoform X1 [KO:K04644]
121453235  Cltb; clathrin light chain B isoform X1 [KO:K04645]
121456200  Cltc; clathrin heavy chain 1 isoform X1 [KO:K04646]
121446016  Hip1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
121457529  Ap2a2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
121440717  Ap2a1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
121456888  Ap2b1; AP-2 complex subunit beta isoform X1 [KO:K11825]
121466494  AP-2 complex subunit beta-like [KO:K11825]
121464793  Ap2m1; AP-2 complex subunit mu [KO:K11826]
121449571  Ap2s1; AP-2 complex subunit sigma [KO:K11827]
121459250  huntingtin isoform X1 [KO:K04533]
121436398  huntingtin-like isoform X1 [KO:K04533]
121437277  Dctn1; dynactin subunit 1 isoform X1 [KO:K04648]
121455956  Dctn2; dynactin subunit 2 isoform X1 [KO:K10424]
121458475  Dctn3; dynactin subunit 3 [KO:K10425]
121445240  dynactin subunit 4 isoform X1 [KO:K10426]
121440645  dynactin subunit 4 isoform X1 [KO:K10426]
121457597  Dctn5; dynactin subunit 5 [KO:K10427]
121462301  Dctn6; dynactin subunit 6 [KO:K10428]
121454258  Actr1a; alpha-centractin isoform X1 [KO:K16575]
121450824  Actr1b; beta-centractin [KO:K16575]
121452083  Actr10; actin-related protein 10 [KO:K16576]
121442465  Hap1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
121455127  Dnah12; dynein heavy chain 12, axonemal [KO:K10408]
121455159  Dnah1; dynein heavy chain 1, axonemal [KO:K10408]
121456109  Dnah2; dynein heavy chain 2, axonemal [KO:K10408]
121456176  Dnah9; dynein heavy chain 9, axonemal [KO:K10408]
121457725  Dnah3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
121458900  Dnah7; dynein heavy chain 7, axonemal [KO:K10408]
121436172  Dnah17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
121445858  Dnah10; dynein heavy chain 10, axonemal [KO:K10408]
121438680  Dnah11; dynein heavy chain 11, axonemal [KO:K10408]
121438736  Dnah6; dynein heavy chain 6, axonemal [KO:K10408]
121433622  Dnah5; dynein heavy chain 5, axonemal [KO:K10408]
121435407  Dnah8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
121435787  Dnah14; dynein heavy chain 14, axonemal [KO:K10408]
121448540  Dnali1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
121458437  Dnai1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409]
121436330  Dnai2; dynein intermediate chain 2, axonemal [KO:K11143]
121465218  tubulin alpha-3 chain-like isoform X1 [KO:K07374]
121465871  tubulin alpha-3 chain [KO:K07374]
121438630  Tuba8; tubulin alpha-8 chain isoform X1 [KO:K07374]
121446953  Tuba4a; tubulin alpha-4A chain [KO:K07374]
121432569  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
121441456  tubulin alpha-1A chain [KO:K07374]
121441457  tubulin alpha-1C chain [KO:K07374]
121441470  Tuba1b; tubulin alpha-1B chain [KO:K07374]
121461780  tubulin beta chain-like [KO:K07375]
121462644  Tubb4b; tubulin beta-4B chain [KO:K07375]
121459334  Tubb4a; tubulin beta-4A chain [KO:K07375]
121437989  LOW QUALITY PROTEIN: tubulin beta-6 chain-like [KO:K07375]
121438749  Tubb6; tubulin beta-6 chain [KO:K07375]
121438763  tubulin beta chain [KO:K07375]
121447276  Tubb3; tubulin beta-3 chain [KO:K07375]
121440863  Tubb1; tubulin beta-1 chain [KO:K07375]
121433933  tubulin beta-2A chain [KO:K07375]
121433980  tubulin beta-2B chain isoform X1 [KO:K07375]
121435417  Tubb; tubulin beta chain [KO:K07375]
121455025  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
121456117  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
121440925  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
121455924  Kif5a; kinesin heavy chain isoform X1 [KO:K10396]
121433144  Kif5c; kinesin heavy chain [KO:K10396]
121440697  Kif5b; kinesin-1 heavy chain isoform X1 [KO:K10396]
121454026  Klc2; kinesin light chain 2 [KO:K10407]
121450493  Klc4; kinesin light chain 4 [KO:K10407]
121434351  Klc1; kinesin light chain 1 isoform X1 [KO:K10407]
121449295  Klc3; LOW QUALITY PROTEIN: kinesin light chain 3 [KO:K10407]
121461776  Ift57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
121458619  caspase-8-like [KO:K04398] [EC:3.4.22.61]
121444834  caspase-8-like [KO:K04398] [EC:3.4.22.61]
121433874  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
121457717  Grm5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
121454687  Gnaq; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
121460541  Plcb4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
121454560  Plcb3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
121465646  Plcb2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
121460033  Plcb1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
121446093  Stx1a; syntaxin-1A [KO:K04560]
121462813  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
121462706  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
121433987  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
121440758  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
121456539  Dlg4; disks large homolog 4 isoform X1 [KO:K11828]
121456665  Gria1; glutamate receptor 1 isoform X1 [KO:K05197]
121452885  Gria2; glutamate receptor 2 isoform X1 [KO:K05198]
121439510  Gria3; glutamate receptor 3 isoform X1 [KO:K05199]
121451688  Gria4; glutamate receptor 4 isoform X1 [KO:K05200]
121460320  Tgm2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
121432952  Slc1a3; excitatory amino acid transporter 1 [KO:K05614]
121465672  Slc1a2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
121442057  Kcnj10; ATP-sensitive inward rectifier potassium channel 10 isoform X1 [KO:K05003]
121460801  Rest; RE1-silencing transcription factor [KO:K09222]
121438235  Sin3a; paired amphipathic helix protein Sin3a [KO:K11644]
121434307  Rcor1; REST corepressor 1 [KO:K11829]
121438918  LOW QUALITY PROTEIN: histone deacetylase 1-like [KO:K06067] [EC:3.5.1.98]
121448269  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
121449324  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
121443315  LOW QUALITY PROTEIN: histone deacetylase 1-like [KO:K06067] [EC:3.5.1.98]
121443415  LOW QUALITY PROTEIN: histone deacetylase 1-like [KO:K06067] [EC:3.5.1.98]
121456325  Polr2a; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
121460717  Polr2b; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
121447105  Polr2c; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
121453473  Polr2d; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
121450655  DNA-directed RNA polymerase II subunit RPB4-like [KO:K03012]
121450656  DNA-directed RNA polymerase II subunit RPB4-like [KO:K03012]
121445752  DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
121459509  Polr2e; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
121461177  Polr2f; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
121454389  Polr2g; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
121465166  Polr2h; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
121449664  Polr2i; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
121457752  Polr2l; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
121446403  Polr2j; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
121459074  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
121453338  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
121437648  Polr2k; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
121465693  Bdnf; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
121461086  histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
121463856  CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
121453272  transcription initiation factor TFIID subunit 4B-like [KO:K03129]
121440868  Taf4; transcription initiation factor TFIID subunit 4 [KO:K03129]
121443157  transcription initiation factor TFIID subunit 4B-like [KO:K03129]
121462906  Tbpl2; TATA box-binding protein-like 2 [KO:K03120]
121466523  TATA-box-binding protein isoform X1 [KO:K03120]
121449284  Tbpl1; TATA box-binding protein-like 1 [KO:K03120]
121459388  transcription factor Sp1-like [KO:K04684]
121444220  transcription factor Sp1 [KO:K04684]
121433487  Creb1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
121463404  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
121458259  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
121459918  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
121450873  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 [KO:K09048]
121437635  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
121439698  Creb5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
121442270  Ppargc1a; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
121437699  Pparg; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
121450901  Tfam; transcription factor A, mitochondrial [KO:K11830]
121463346  Nrf1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
121465280  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
121446310  superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
121466392  Sod2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
121461230  glutathione peroxidase 1-like [KO:K00432] [EC:1.11.1.9]
121455863  Gpx8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
121456425  Gpx3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
121464326  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
121452293  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
121443961  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
121443964  Gpx5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
121448516  Gpx7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
121455936  Ucp1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
121456449  Tp53; cellular tumor antigen p53 isoform X1 [KO:K04451]
121449799  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
121449455  Bbc3; bcl-2-binding component 3 isoform X1 [KO:K10132]
121454797  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
121433544  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
121435615  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
121441232  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial-like [KO:K03944]
121441356  NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial-like [KO:K03944]
121444726  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
121441690  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
121453388  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
121443005  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
121461094  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
121452542  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
121436655  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
121437058  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
121434833  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
121450172  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
121462117  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
121463391  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
121437134  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
121440527  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
121463343  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
121461352  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
121459203  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
121462900  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
121442925  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
121447625  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
121433540  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
121457745  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
121453551  acyl carrier protein, mitochondrial-like [KO:K03955]
121442253  acyl carrier protein, mitochondrial-like [KO:K03955]
121459644  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
121460867  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
121462835  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
121443386  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
121434340  Ndufb1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
121463417  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
121458750  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
121435543  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
121458872  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
121444862  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
121461845  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
121446614  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
121452806  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
121458387  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
121455892  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
121454191  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
121461070  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
121463796  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
121443686  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
121443701  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
121443711  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
121443746  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
121443749  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial-like [KO:K11351]
121433414  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
121455075  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
121442040  NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
121437628  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
121455986  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
121465282  NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
121455508  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
121448570  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
121433689  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
121459779  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
121454295  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
121452875  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
121452979  NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967]
121458965  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
121449322  NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968]
121433679  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
121448291  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
121455251  succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
121442082  succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
121438209  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
121434112  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
121461124  cytochrome c1, heme protein, mitochondrial [KO:K00413]
121464397  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
121458092  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
121443904  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
121448425  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
121437672  cytochrome b-c1 complex subunit 7 [KO:K00417]
121439775  cytochrome b-c1 complex subunit 7-like [KO:K00417]
121433101  cytochrome b-c1 complex subunit 7-like [KO:K00417]
121457156  cytochrome b-c1 complex subunit 8 [KO:K00418]
121435761  cytochrome b-c1 complex subunit 8-like [KO:K00418]
121459730  cytochrome b-c1 complex subunit 9 [KO:K00419]
121436552  cytochrome b-c1 complex subunit 9 [KO:K00419]
121459229  cytochrome b-c1 complex subunit 10 [KO:K00420]
121438432  cytochrome b-c1 complex subunit 10-like [KO:K00420]
121434846  cytochrome b-c1 complex subunit 10-like [KO:K00420]
121435876  cytochrome b-c1 complex subunit 10 [KO:K00420]
121460449  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
121446836  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
121445365  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
121450860  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
121457523  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
121444805  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
121449638  cytochrome c oxidase subunit 6B1 [KO:K02267]
121450077  cytochrome c oxidase subunit 6B2 [KO:K02267]
121441749  cytochrome c oxidase subunit 6B1-like [KO:K02267]
121448170  cytochrome c oxidase subunit 6B1-like [KO:K02267]
121437587  cytochrome c oxidase subunit 6C-2 [KO:K02268]
121459089  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
121466093  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
121446603  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
121432415  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
121432956  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
121434798  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
121449666  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
121460628  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
121457965  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
121465057  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
121444814  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
121451002  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
121442733  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
121455767  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
121460409  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
121441397  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
121454314  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
121463225  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
121458083  cytochrome c oxidase subunit 8B, mitochondrial-like [KO:K02273]
121434483  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
121460327  ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
121440152  Atp5f1a; ATP synthase subunit alpha, mitochondrial [KO:K02132]
121462537  Atp5f1b; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
121432614  Atp5f1c; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
121459196  Atp5f1d; ATP synthase subunit delta, mitochondrial [KO:K02134]
121440867  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
121461647  ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
121452259  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
121438065  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
121435793  ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
121455306  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
121455786  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
121456597  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
121463377  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121458223  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121459624  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
121466155  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
121443967  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121437003  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121439095  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
121439363  ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
121447428  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121433699  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121441511  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
121442074  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
121454949  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121454957  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121459040  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121436220  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
121439953  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121447366  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121434729  ATP synthase subunit d, mitochondrial-like [KO:K02138]
121461652  Atp5po; ATP synthase subunit O, mitochondrial [KO:K02137]
121465330  Atp5pf; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
121456504  Vdac1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
121438629  voltage-dependent anion-selective channel protein 2 [KO:K15040]
121435027  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
121462373  Vdac3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
121452943  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
121451521  ADP/ATP translocase 2-like [KO:K05863]
121451756  ADP/ATP translocase 2 [KO:K05863]
121444701  ADP/ATP translocase 2 [KO:K05863]
121433765  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
121441648  ADP/ATP translocase 2 [KO:K05863]
121435628  ADP/ATP translocase 2-like [KO:K05863]
121455138  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
121454900  cytochrome c, somatic-like [KO:K08738]
121455103  cytochrome c, somatic [KO:K08738]
121455788  cytochrome c, somatic-like [KO:K08738]
121463471  cytochrome c, somatic-like [KO:K08738]
121446193  cytochrome c, somatic-like [KO:K08738]
121439072  cytochrome c, testis-specific [KO:K08738]
121439303  cytochrome c, testis-specific [KO:K08738]
121439899  cytochrome c, somatic [KO:K08738]
121434736  cytochrome c, somatic [KO:K08738]
121435586  cytochrome c, somatic-like [KO:K08738]
121462949  Apaf1; apoptotic protease-activating factor 1 [KO:K02084]
121448746  Casp9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
121452326  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
121455897  proteasome subunit alpha type-2-like [KO:K02726] [EC:3.4.25.1]
121432751  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
121438357  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
121459573  proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
121453636  proteasome subunit alpha type-8 [KO:K02731] [EC:3.4.25.1]
121440802  Psma7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
121443070  proteasome subunit alpha type-8-like isoform X1 [KO:K02731] [EC:3.4.25.1]
121443166  proteasome subunit alpha type-8 [KO:K02731] [EC:3.4.25.1]
121452594  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
121454266  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
121457771  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
121452345  Psma3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
121456544  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
121462585  proteasome subunit beta type-7-like [KO:K02739] [EC:3.4.25.1]
121462592  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
121456729  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
121444975  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
121438020  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
121442459  proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
121455247  proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
121448409  Psmb2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
121439600  proteasome subunit beta type-5-like [KO:K02737] [EC:3.4.25.1]
121434997  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
121466524  Psmb1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
121440539  proteasome subunit beta type-1-like [KO:K02732] [EC:3.4.25.1]
121450882  proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
121438002  proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
121458903  Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061]
121434726  26S proteasome regulatory subunit 7 [KO:K03061]
121439032  26S proteasome regulatory subunit 4 [KO:K03062]
121434480  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
121449345  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
121435140  Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064]
121437637  Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065]
121436184  Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066]
121465086  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
121447132  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
121457080  26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
121442497  26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
121444166  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
121436267  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
121456681  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
121455154  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
121447595  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
121458035  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
121450463  26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
121437836  26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
121433293  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
121449470  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
121440800  Adrm1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
121449139  Map3k10; mitogen-activated protein kinase kinase kinase 10 isoform X1 [KO:K04418] [EC:2.7.11.25]
121436130  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
121463096  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
121449186  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
121464926  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
121448014  serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
121441746  Mtor; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
121446066  Ulk1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
121456068  Ulk2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
121444254  Atg101; autophagy-related protein 101 [KO:K19730]
121437610  Atg13; autophagy-related protein 13 isoform X1 [KO:K08331]
121449319  autophagy-related protein 13-like [KO:K08331]
121451285  Rb1cc1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
121437617  Ambra1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
121442443  Becn1; beclin-1 isoform X1 [KO:K08334]
121464448  Pik3r4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
121435054  Atg14; beclin 1-associated autophagy-related key regulator isoform X1 [KO:K17889]
121450669  Nrbf2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
121453313  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
121441902  phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
121454280  Atg2a; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
121434320  Atg2b; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
121464893  WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
121446026  WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
121436053  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
morg00190  Oxidative phosphorylation
morg03020  RNA polymerase
morg03022  Basal transcription factors
morg03050  Proteasome
morg04020  Calcium signaling pathway
morg04115  p53 signaling pathway
morg04140  Autophagy - animal
morg04141  Protein processing in endoplasmic reticulum
morg04144  Endocytosis
morg04210  Apoptosis
morg04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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