KEGG   PATHWAY: mpah05016
Entry
mpah05016                   Pathway                                
Name
Huntington disease - Mus pahari (shrew mouse)
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
mpah05016  Huntington disease
mpah05016

Organism
Mus pahari (shrew mouse) [GN:mpah]
Gene
110338632  Clta; clathrin light chain A isoform X1 [KO:K04644]
110333789  Cltb; clathrin light chain B isoform X1 [KO:K04645]
110332091  Cltc; clathrin heavy chain 1 isoform X1 [KO:K04646]
110313165  Hip1; huntingtin-interacting protein 1 [KO:K04559]
110316055  Ap2a1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
110335445  Ap2a2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
110331191  Ap2b1; AP-2 complex subunit beta [KO:K11825]
110329326  Ap2m1; AP-2 complex subunit mu isoform X1 [KO:K11826]
110336628  Ap2s1; AP-2 complex subunit sigma [KO:K11827]
110330941  Htt; huntingtin [KO:K04533]
110313744  Dctn1; dynactin subunit 1 isoform X1 [KO:K04648]
110326429  Dctn2; dynactin subunit 2 isoform X1 [KO:K10424]
110338650  Dctn3; dynactin subunit 3 isoform X1 [KO:K10425]
110332921  Dctn4; dynactin subunit 4 isoform X1 [KO:K10426]
110319336  Dctn5; dynactin subunit 5 [KO:K10427]
110336712  Dctn6; dynactin subunit 6 [KO:K10428]
110317024  Actr1a; alpha-centractin isoform X1 [KO:K16575]
110321668  Actr1b; beta-centractin [KO:K16575]
110324388  Actr10; actin-related protein 10 [KO:K16576]
110331682  Hap1; huntingtin-associated protein 1 [KO:K04647]
110332053  Dnah2; LOW QUALITY PROTEIN: dynein heavy chain 2, axonemal [KO:K10408]
110316435  Dnah6; dynein heavy chain 6, axonemal [KO:K10408]
110325230  Dnah1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
110328833  Dnah5; dynein heavy chain 5, axonemal [KO:K10408]
110324139  Dnah11; dynein heavy chain 11, axonemal [KO:K10408]
110324142  Dnah3; dynein heavy chain 3, axonemal [KO:K10408]
110322079  Dnah14; dynein heavy chain 14, axonemal [KO:K10408]
110325731  Dnah12; dynein heavy chain 12, axonemal [KO:K10408]
110332412  Dnah17; dynein heavy chain 17, axonemal isoform X1 [KO:K10408]
110332474  Dnah9; dynein heavy chain 9, axonemal [KO:K10408]
110338515  Dnah8; dynein heavy chain 8, axonemal [KO:K10408]
110313027  Dnah10; dynein heavy chain 10, axonemal [KO:K10408]
110322936  Dnali1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
110338758  Dnai1; dynein intermediate chain 1, axonemal [KO:K10409]
110331888  Dnai2; dynein intermediate chain 2, axonemal [KO:K11143]
110324749  Dnal1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
110334885  Dnal4; dynein light chain 4, axonemal [KO:K10412]
110316306  tubulin alpha-3 chain isoform X1 [KO:K07374]
110321284  Tuba4a; tubulin alpha-4A chain isoform X1 [KO:K07374]
110334563  tubulin alpha-1C chain-like [KO:K07374]
110316395  Tuba8; tubulin alpha-8 chain isoform X1 [KO:K07374]
110316417  tubulin alpha-3 chain isoform X1 [KO:K07374]
110334627  tubulin alpha-1A chain-like [KO:K07374]
110325235  tubulin alpha chain-like [KO:K07374]
110334021  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
110319104  Tubb1; tubulin beta-1 chain [KO:K07375]
110333011  Tubb6; tubulin beta-6 chain isoform X1 [KO:K07375]
110335873  Tubb; tubulin beta chain isoform X1 [KO:K07375]
110335384  Tubb4a; tubulin beta-4A chain isoform X1 [KO:K07375]
110337799  Tubb3; tubulin beta-3 chain isoform X1 [KO:K07375]
110333774  tubulin beta-2A chain-like [KO:K07375]
110319452  Tubb4b; tubulin beta-4B chain isoform X1 [KO:K07375]
110333829  LOW QUALITY PROTEIN: tubulin beta-2B chain-like [KO:K07375]
110330449  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
110332652  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
110325466  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
110332910  Kif5b; kinesin-1 heavy chain [KO:K10396]
110326230  Kif5a; kinesin heavy chain [KO:K10396]
110318104  Kif5c; kinesin heavy chain [KO:K10396]
110319541  Klc2; kinesin light chain 2 [KO:K10407]
110336348  Klc3; kinesin light chain 3 isoform X1 [KO:K10407]
110324984  Klc1; kinesin light chain 1 isoform X1 [KO:K10407]
110335434  Klc4; kinesin light chain 4 isoform X1 [KO:K10407]
110329653  Ift57; intraflagellar transport protein 57 homolog [KO:K04638]
110321675  Casp8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
110336608  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
110318463  Grm5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
110336980  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
110336671  Plcb3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
110317815  Plcb4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 [KO:K05858] [EC:3.1.4.11]
110318415  Plcb2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
110319646  Plcb1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
110339108  Stx1a; syntaxin-1A [KO:K04560]
110318209  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
110319261  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
110316284  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
110317210  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
110332219  Dlg4; disks large homolog 4 isoform X1 [KO:K11828]
110331056  Gria1; glutamate receptor 1 isoform X1 [KO:K05197]
110320188  Gria2; glutamate receptor 2 isoform X1 [KO:K05198]
110314078  Gria3; glutamate receptor 3 isoform X1 [KO:K05199]
110327159  Gria4; glutamate receptor 4 isoform X1 [KO:K05200]
110317548  Tgm2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
110328755  Slc1a3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
110317544  Slc1a2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
110322293  Kcnj10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
110330594  Rest; RE1-silencing transcription factor [KO:K09222]
110328718  Sin3a; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
110324432  Rcor1; REST corepressor 1 [KO:K11829]
110326229  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
110322864  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
110332723  Polr2a; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
110330235  Polr2b; DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
110337408  Polr2c; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
110333300  Polr2d; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
110326739  Polr2e; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
110334774  Polr2f; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
110327913  Polr2g; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
110329787  Polr2h; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
110323026  Polr2i; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
110316393  Polr2l; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
110336652  DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
110312947  Polr2j; DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
110335171  Polr2k; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
110318089  Bdnf; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
110334526  Ep300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
110330048  Crebbp; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
110333347  Taf4b; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
110318080  Taf4; transcription initiation factor TFIID subunit 4 [KO:K03129]
110338047  Tbp; TATA-box-binding protein isoform X1 [KO:K03120]
110319015  Tbpl2; TATA box-binding protein-like protein 2 isoform X1 [KO:K03120]
110338035  Tbpl1; TATA box-binding protein-like protein 1 [KO:K03120]
110334488  Sp1; transcription factor Sp1 isoform X1 [KO:K04684]
110322391  Creb1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
110320678  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
110316152  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
110326077  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
110338975  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
110318364  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
110315994  Creb5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
110330452  Ppargc1a; peroxisome proliferator-activated receptor gamma coactivator 1-alpha [KO:K07202]
110317409  Pparg; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
110326529  Tfam; transcription factor A, mitochondrial [KO:K11830]
110315399  Nrf1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
110329810  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
110338019  Sod2; superoxide dismutase [Mn], mitochondrial isoform X1 [KO:K04564] [EC:1.15.1.1]
110324341  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
110327909  Gpx1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
110334041  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
110323454  Gpx7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
110333985  Gpx5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
110332058  Gpx3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
110329021  Gpx8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
110337649  Ucp1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
110331731  Tp53; cellular tumor antigen p53 isoform X1 [KO:K04451]
110320030  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
110336584  Bbc3; bcl-2-binding component 3 [KO:K10132]
36165356  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
36165352  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
36165337  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
36165334  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
36165335  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
36165330  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
36165329  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
110316888  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
110335628  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
110338235  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
110313685  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
110333203  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
110337062  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
110326234  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
110317006  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
110316386  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
110335084  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
110335777  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
110318772  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
110316704  Ndufa9; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
110321748  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
110328243  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
110335867  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
110326784  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
110336643  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
110324555  Ndufb1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
110317149  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
110322308  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
110326037  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
110329959  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
110320492  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
110338914  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
110333533  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6-like [KO:K03962]
110337650  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
110331668  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
110334970  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
110338345  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
110314570  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
110321204  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
110321406  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
110318572  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
110329019  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
110323693  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
110329167  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
110326881  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
110327359  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
110320722  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
110332408  NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968]
110328746  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
110323139  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial isoform X1 [KO:K00235] [EC:1.3.5.1]
110322178  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
110324197  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like isoform X1 [KO:K00237]
110328112  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
110333870  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
36165327  CYTB; cytochrome b [KO:K00412]
110334724  cytochrome c1, heme protein, mitochondrial [KO:K00413]
110327331  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
110318232  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
110323660  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
110334014  cytochrome b-c1 complex subunit 7 [KO:K00417]
110332489  cytochrome b-c1 complex subunit 8 [KO:K00418]
110330872  cytochrome b-c1 complex subunit 9 [KO:K00419]
110326979  cytochrome b-c1 complex subunit 10 [KO:K00420]
36165339  COX3; cytochrome c oxidase subunit III [KO:K02262]
36165346  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
36165343  COX2; cytochrome c oxidase subunit II [KO:K02261]
110319299  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
110337554  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X1 [KO:K02263]
110328046  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
110322258  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
110313018  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
110325548  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
110332943  cytochrome c oxidase subunit 6B1 [KO:K02267]
110336798  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
110335202  cytochrome c oxidase subunit 6C [KO:K02268]
110328169  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
110338814  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
110335711  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
110313979  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
110330226  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
110329085  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
110332346  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
110324960  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
110339130  cytochrome c oxidase subunit 8B, mitochondrial [KO:K02273]
110333376  Atp5a1; ATP synthase subunit alpha, mitochondrial isoform X1 [KO:K02132]
110324657  ATP synthase subunit beta, mitochondrial-like [KO:K02133] [EC:7.1.2.2]
110326266  Atp5b; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
110333647  Atp5c1; ATP synthase subunit gamma, mitochondrial [KO:K02136]
110326670  Atp5d; ATP synthase subunit delta, mitochondrial isoform X1 [KO:K02134]
110319359  Atp5e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
36165340  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
110320209  Atp5f1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
110318554  Atp5g3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128]
110331847  Atp5g1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
110334965  Atp5g2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
110320480  ATP synthase subunit d, mitochondrial-like isoform X1 [KO:K02138]
110332361  Atp5h; ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138]
110329905  Atp5o; ATP synthase subunit O, mitochondrial [KO:K02137]
110329594  Atp5j; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
36165341  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
110331342  Vdac1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
110325370  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
110336305  Vdac3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
110313362  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
110336417  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
110320569  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
110325751  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
110332378  cytochrome c-like [KO:K08738]
110316223  cytochrome c, somatic [KO:K08738]
110317786  cytochrome c, testis-specific [KO:K08738]
110326877  Apaf1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
110323338  Casp9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
110324196  Psma6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
110333899  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
110327417  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
110319566  Psma7; proteasome subunit alpha type-7 isoform X1 [KO:K02731] [EC:3.4.25.1]
110333269  Psma8; proteasome subunit alpha type-7-like isoform X1 [KO:K02731] [EC:3.4.25.1]
110320113  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
110321258  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
110324319  Psma3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
110331969  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
110317854  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
110332263  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
110323478  Psmb2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
110325463  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
110338158  Psmb1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
110320096  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
110317419  Psmc2; 26S protease regulatory subunit 7 [KO:K03061]
110324155  Psmc1; 26S protease regulatory subunit 4 isoform X1 [KO:K03062]
110317526  Psmc4; 26S protease regulatory subunit 6B isoform X1 [KO:K03063]
110325226  Psmc6; 26S protease regulatory subunit 10B [KO:K03064]
110317665  Psmc3; 26S protease regulatory subunit 6A [KO:K03065]
110331458  Psmc5; 26S protease regulatory subunit 8 [KO:K03066]
110329924  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 isoform X1 [KO:K03028]
110321309  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
110332775  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
110312964  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
110331343  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
110331208  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
110325295  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
110337398  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
110319108  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
110318110  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
110323088  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
110318228  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
110319004  proteasomal ubiquitin receptor ADRM1-like [KO:K06691]
110317098  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
110327052  Map3k10; mitogen-activated protein kinase kinase kinase 10 isoform X1 [KO:K04418] [EC:2.7.11.25]
110331346  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
110318405  Traf2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
110337870  Map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
110336400  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
110323363  Mtor; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
110339107  Ulk1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
110331327  Ulk2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
110334989  Atg101; autophagy-related protein 101 [KO:K19730]
110317587  Atg13; autophagy-related protein 13 isoform X1 [KO:K08331]
110338658  Rb1cc1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
110317534  Ambra1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
110331121  Becn1; beclin-1 isoform X1 [KO:K08334]
110327680  Pik3r4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
110325448  Atg14; beclin 1-associated autophagy-related key regulator [KO:K17889]
110326783  Nrbf2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
110332837  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
110324091  Atg2b; autophagy-related protein 2 homolog B [KO:K17906]
110330064  Atg2a; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
110331591  Wipi1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
110339041  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
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La Spada AR, Morrison RS
  Title
The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
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Neuron 47:1-3 (2005)
DOI:10.1016/j.neuron.2005.06.023
Reference
PMID:11738471
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Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
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Neurochem Int 40:37-52 (2002)
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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
mpah00190  Oxidative phosphorylation
mpah03020  RNA polymerase
mpah03022  Basal transcription factors
mpah03050  Proteasome
mpah04020  Calcium signaling pathway
mpah04115  p53 signaling pathway
mpah04140  Autophagy - animal
mpah04141  Protein processing in endoplasmic reticulum
mpah04144  Endocytosis
mpah04210  Apoptosis
mpah04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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