KEGG   PATHWAY: rno05016
Entry
rno05016                    Pathway                                
Name
Huntington disease - Rattus norvegicus (rat)
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
rno05016  Huntington disease
rno05016

Disease
H00059  Huntington disease
Organism
Rattus norvegicus (rat) [GN:rno]
Gene
100188937  Cox7c; cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
100360357  Cox7cl1; cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
100360846  Psmb6l1; proteasome 20S subunit beta 6 like 1 [KO:K02738] [EC:3.4.25.1]
100361008  [KO:K02264]
100361574  Polr2k; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
100361833  Atp5mc2l1; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100361879  [KO:K02135]
100361934  Ndufb4l4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
100361960  Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271]
100362331  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
100362798  Uqcr11l1; cytochrome b-c1 complex subunit 10-like [KO:K00420]
100363472  [KO:K21246]
100911483  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100912357  Ndufb1; nADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
102554884  Rcor1; REST corepressor 1 [KO:K11829]
103690609  Ndufb3l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
103691107  Ndufb4l5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
103691697  Ndufb3l2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
103693740  Uqcrbl2; cytochrome b-c1 complex subunit 7-like [KO:K00417]
103694107  Ndufb3l3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
113919  Gpx5; epididymal secretory glutathione peroxidase precursor [KO:K00432] [EC:1.11.1.9]
114558  Becn1; beclin-1 [KO:K08334]
114630  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial precursor [KO:K02128]
116470  Stx1a; syntaxin-1A [KO:K04560]
116550  Atp5f1c; ATP synthase subunit gamma, mitochondrial [KO:K02136]
116554  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
116561  Cltb; clathrin light chain B [KO:K04645]
116563  Ap2m1; AP-2 complex subunit mu [KO:K11826]
117017  Polr2g; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
117249  Dnah3; dynein axonemal heavy chain 3 isoform X1 [KO:K10408]
117250  Dnah6; dynein axonemal heavy chain 6 [KO:K10408]
117251  Dnah9; dynein, axonemal, heavy chain 9 [KO:K10408]
117252  Dnah10; dynein axonemal heavy chain 10 isoform X1 [KO:K10408]
117253  Dnah11; dynein axonemal heavy chain 11 isoform X2 [KO:K10408]
117262  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
117263  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
117526  Tbp; TATA-box-binding protein [KO:K03120]
117550  Kif5b; kinesin-1 heavy chain [KO:K10396] [EC:5.6.1.3]
120094308  [KO:K03960]
120095266  [KO:K02272]
120097699  [KO:K00420]
120098258  [KO:K02268]
120098305  [KO:K03016]
120098314  [KO:K02128]
120098318  [KO:K03959]
120099907  [KO:K03946]
120099910  [KO:K08738]
120101567  [KO:K03951]
120103152  [KO:K02267]
140670  Ap2b1; AP-2 complex subunit beta [KO:K11825]
157074  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial precursor [KO:K00234] [EC:1.3.5.1]
161475  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
170915  Ep300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
171041  Klc1; kinesin light chain 1 isoform C [KO:K10407]
171082  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial precursor [KO:K02128]
171335  Cox8a; cytochrome c oxidase subunit 8A, mitochondrial precursor [KO:K02273]
171339  Dnah1; dynein axonemal heavy chain 1 [KO:K10408]
171374  Atp5f1b; ATP synthase subunit beta, mitochondrial precursor [KO:K02133] [EC:7.1.2.2]
171375  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial precursor [KO:K02127]
171549  Klc3; kinesin light chain 3 [KO:K10407]
192154  Hip1; huntingtin-interacting protein 1 isoform 2 [KO:K04559]
192241  Atp5po; ATP synthase subunit O, mitochondrial precursor [KO:K02137]
207117  Dnah8; dynein heavy chain 8, axonemal [KO:K10408]
24225  Bdnf; brain-derived neurotrophic factor isoform 3 preproprotein [KO:K04355]
24404  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
24408  Grin1; glutamate receptor ionotropic, NMDA 1 isoform 1a precursor [KO:K05208]
24410  Grin2b; glutamate receptor ionotropic, NMDA 2B precursor [KO:K05210]
24418  Grm5; metabotropic glutamate receptor 5 precursor [KO:K04604]
245958  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
245965  Atp5f1d; ATP synthase subunit delta, mitochondrial precursor [KO:K02134]
246118  Tubb3; tubulin beta-3 chain [KO:K07375]
24654  Plcb1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
24786  Sod1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
24787  Sod2; superoxide dismutase [Mn], mitochondrial precursor [KO:K04564] [EC:1.15.1.1]
24790  Sp1; transcription factor Sp1 [KO:K04684]
24842  Tp53; cellular tumor antigen p53 [KO:K04451]
24860  Ucp1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
24887  Bax; apoptosis regulator BAX [KO:K02159]
25031  Plcb4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 [KO:K05858] [EC:3.1.4.11]
25176  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
25250  Cox8b; cytochrome c oxidase subunit 8B, mitochondrial precursor [KO:K02273]
25262  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform 3 [KO:K04958]
25272  Mapk10; mitogen-activated protein kinase 10 isoform 2 [KO:K04440] [EC:2.7.11.24]
25278  Cox6a2; cytochrome c oxidase subunit 6A2, mitochondrial precursor [KO:K02266]
25282  Cox6a1; cytochrome c oxidase subunit 6A1, mitochondrial precursor [KO:K02266]
252893  Dnah7; dynein axonemal heavy chain 7 [KO:K10408]
252934  Cox5a; cytochrome c oxidase subunit 5A, mitochondrial precursor [KO:K02264]
252959  Dnah12; dynein axonemal heavy chain 12 [KO:K10408]
25309  Cycs; cytochrome c, somatic [KO:K08738]
25310  Cyct; cytochrome c, testis-specific [KO:K08738]
25402  Casp3; caspase-3 [KO:K02187] [EC:3.4.22.56]
25488  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
25581  Psmc2; 26S proteasome regulatory subunit 7 [KO:K03061]
25664  Pparg; peroxisome proliferator-activated receptor gamma isoform 1 [KO:K08530]
257648  Cacna1b; voltage-dependent N-type calcium channel subunit alpha-1B isoform 1 [KO:K04849]
259233  Gpx6; glutathione peroxidase 6 precursor [KO:K00432] [EC:1.11.1.9]
26192  CYTB; cytochrome b [KO:K00412]
26193  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
26194  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
26195  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
26196  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
26197  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
26198  COX2; cytochrome c oxidase subunit II [KO:K02261]
26199  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
26200  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
26201  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
26202  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
26203  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
26204  COX3; cytochrome c oxidase subunit III [KO:K02262]
282819  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial precursor [KO:K09565] [EC:5.2.1.8]
287670  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
287772  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
287845  Dnah17; dynein axonemal heavy chain 17 [KO:K10408]
287984  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
288088  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
288498  Wipi2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
288588  Polr2j; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
289217  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
289218  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial precursor [KO:K03935] [EC:7.1.1.2]
289561  Polr2b; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
289924  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
289990  Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064]
290019  Cycsl3; cytochrome c, somatic-like [KO:K08738]
290798  Dctn6; dynactin subunit 6 [KO:K10428]
291081  Tubb2b; tubulin beta-2B chain [KO:K07375]
291103  Uqcrfs1; cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
291287  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
291660  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
29167  Dctn1; dynactin subunit 1 [KO:K04648]
291773  Taf4b; transcription initiation factor TFIID subunit 4B [KO:K03129]
29213  Tubb4a; tubulin beta-4A chain [KO:K07375]
29214  Tubb5; tubulin beta-5 chain [KO:K07375]
292766  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
292778  Polr2i; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
293130  Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
29322  Plcb3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
29326  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
293448  Uqcrc2; cytochrome b-c1 complex subunit 2, mitochondrial precursor [KO:K00415]
293453  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
293652  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
293655  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
293991  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
294010  Actr1a; alpha-centractin [KO:K16575]
29424  Htt; huntingtin [KO:K04533]
29425  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
29430  Hap1; huntingtin-associated protein 1 isoform B [KO:K04647]
29445  Cox4i1; cytochrome c oxidase subunit 4 isoform 1, mitochondrial precursor [KO:K02263]
294744  Gpx8; probable glutathione peroxidase 8 [KO:K00432] [EC:1.11.1.9]
29482  Slc1a2; excitatory amino acid transporter 2 isoform a [KO:K05613]
29483  Slc1a3; excitatory amino acid transporter 1 isoform 1 [KO:K05614]
294854  Dnah5; dynein axonemal heavy chain 5 [KO:K10408]
29495  Dlg4; disks large homolog 4 [KO:K11828]
294964  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
29507  Cox7a2; cytochrome c oxidase subunit 7A2, mitochondrial precursor [KO:K02270]
295923  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
296169  Psmb3l2; proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
29627  Gria2; glutamate receptor 2 isoform 2 precursor [KO:K05198]
29628  Gria3; glutamate receptor 3 isoform 2 precursor [KO:K05199]
29629  Gria4; glutamate receptor 4 isoform 2 precursor [KO:K05200]
296554  Tubb4b; tubulin beta-4B chain [KO:K07375]
296658  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
29666  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
29668  Psma1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
29669  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
29670  Psma3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
29671  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
29672  Psma5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
29673  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
29674  Psma7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
29675  Psmb2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
29676  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
29677  Psmc3; 26S proteasome regulatory subunit 6A [KO:K03065]
29718  Kcnj10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
29754  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial precursor [KO:K02128]
297893  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
297990  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
298376  Gpx7; glutathione peroxidase 7 precursor [KO:K00432] [EC:1.11.1.9]
298400  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
298524  Dnali1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
298596  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial precursor [KO:K00235] [EC:1.3.5.1]
298762  Cox7a2l; cytochrome c oxidase subunit 7A2 like isoform 1 [KO:K02270]
299121  Actr10; actin-related protein 10 [KO:K16576]
299310  Ndufb11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
299643  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
299739  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
299850  Dctn2; dynactin subunit 2 [KO:K10424]
299954  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
300047  Cyc1; cytochrome c-1 [KO:K00413]
300078  Dnal4; dynein axonemal light chain 4 [KO:K10412]
300218  Tuba1c; tubulin alpha-1C chain [KO:K07374]
300240  Atg101; autophagy-related protein 101 isoform 2 [KO:K19730]
301011  Uqcrc1; cytochrome b-c1 complex subunit 1, mitochondrial precursor [KO:K00414]
301123  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
301427  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
301458  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial precursor [KO:K03934] [EC:7.1.1.2]
303206  Ulk2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
303242  Dnah2; dynein axonemal heavy chain 2 [KO:K10408]
303353  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
303393  Cox7b; cytochrome c oxidase subunit 7B, mitochondrial precursor [KO:K02271]
303630  Wipi1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
303968  Ift57; intraflagellar transport protein 57 homolog [KO:K04638]
305831  Atg14; beclin 1-associated autophagy-related key regulator [KO:K17889]
307351  Tubb6; tubulin beta-6 chain [KO:K07375]
307821  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
308463  Map3k10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
308578  Ap2a1; AP-2 complex subunit alpha-1 isoform 1 [KO:K11824]
308961  Dctn5; dynactin subunit 5 [KO:K10427]
309159  Klc2; kinesin light chain 2 [KO:K10407]
310616  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform 1 [KO:K09048]
311024  Kif5c; kinesin heavy chain isoform 5C [KO:K10396] [EC:5.6.1.3]
311078  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
311786  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
312195  Nrf1; nuclear respiratory factor 1 [KO:K11831]
312927  Rb1cc1; RB1-inducible coiled-coil protein 1 [KO:K17589]
314415  Atg2b; autophagy related 2B [KO:K17906]
314638  Creb3l3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
314906  Kif5a; kinesin heavy chain isoform 5A [KO:K10396] [EC:5.6.1.3]
315167  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
316226  Klc4; kinesin light chain 4 [KO:K10407]
316333  Actr1b; beta-centractin [KO:K16575]
316531  Tuba4a; tubulin alpha-4A chain [KO:K07374]
316632  Ndufa10l1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10-like [KO:K03954]
317673  Bbc3; bcl-2-binding component 3 [KO:K10132]
360229  Cox8c; cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
360654  Dnai2; dynein axonemal intermediate chain 2 [KO:K11143]
360827  Ulk1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
361365  Polr2c; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
361385  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
362164  Atg13; autophagy-related protein 13 [KO:K08331]
362165  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
362339  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
362344  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
362440  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
362504  Dctn3; dynactin subunit 3 [KO:K10425]
362588  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
362837  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
362897  Uqcrb; cytochrome b-c1 complex subunit 7 [KO:K00417]
363061  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
363067  Sin3a; paired amphipathic helix protein Sin3a [KO:K11644]
363131  Pik3r4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
363441  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
363633  Polr2a; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
363855  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform 1 [KO:K04431] [EC:2.7.12.2]
364814  Psma8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
364834  Polr2d; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
365057  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
365388  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
366448  Uqcrh; cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
366614  Atp5mc1l2; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
367149  Bax-ps1; apoptosis regulator BAX-like [KO:K02159]
497902  Uqcrq; cytochrome b-c1 complex subunit 8 [KO:K00418]
498013  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
498109  Polr2h; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
498736  Tubb2a; tubulin beta-2A chain [KO:K07375]
498885  Slc25a6; solute carrier family 25 member 6 [KO:K05863]
498977  Dctn3l1; dynactin 3-like 1 [KO:K10425]
499131  Ndufb4l3; similar to NADH:ubiquinone oxidoreductase B15 subunit [KO:K03960]
499512  Atp5mc2l2; ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
499529  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial precursor [KO:K03937]
499670  RGD1560263; nuclear receptor-binding factor 2-like isoform X1 [KO:K21246]
500131  Creb5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
500319  Tuba3a; tubulin alpha-3 chain [KO:K07374]
500363  Tuba3b; tubulin alpha-3 chain [KO:K07374]
500377  Tuba8; tubulin alpha-8 chain [KO:K07374]
500442  Dnai1; dynein axonemal intermediate chain 1 [KO:K10409]
500871  Slc25a5l1; ADP/ATP translocase 2-like [KO:K05863]
500929  Tuba1b; tubulin alpha-1B chain [KO:K07374]
501886  Ndufb4l1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
502374  Polr2l; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
50592  Gria1; glutamate receptor 1 precursor [KO:K05197]
50658  Mapk9; mitogen-activated protein kinase 9 isoform 2 [KO:K04440] [EC:2.7.11.24]
54241  Cltc; clathrin heavy chain 1 [KO:K04646]
54244  Crebbp; histone lysine acetyltransferase CREBBP [KO:K04498] [EC:2.3.1.48]
54322  Cox6c; cytochrome c oxidase subunit 6C-2 [KO:K02268]
56083  Tgm2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
56718  Mtor; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
58839  Nrbf2; nuclear receptor-binding factor 2 [KO:K21246]
58854  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
58918  Casp9; caspase-9 [KO:K04399] [EC:3.4.22.62]
59319  Ambra1; activating molecule in BECN1-regulated autophagy protein 1 [KO:K17985]
64044  Casp8; caspase-8 [KO:K04398] [EC:3.4.22.61]
641434  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
64158  Tuba1a; tubulin alpha-1A chain [KO:K07374]
64317  Gpx3; glutathione peroxidase 3 precursor [KO:K00432] [EC:1.11.1.9]
64539  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform 2 [KO:K03944]
65046  Ap2s1; AP-2 complex subunit sigma [KO:K11827]
65052  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
65138  Adrm1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
65262  Atp5f1a; ATP synthase subunit alpha, mitochondrial precursor [KO:K02132]
654441  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
678759  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial precursor [KO:K03954]
679312  Tubb1; tubulin beta-1 chain isoform X1 [KO:K07375]
679739  Ndufs6; NADH:ubiquinone oxidoreductase subunit S6-like 1 [KO:K03939]
679794  Cycsl1; cytochrome c, somatic-like [KO:K08738]
680050  Tbpl2; TATA box-binding protein-like 2 [KO:K03120]
680532  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
681024  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
681193  Polr2sl1; DNA-directed RNA polymerases I, II, and III subunit RPABC5-like [KO:K03007]
681314  [KO:K02159]
681418  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
682097  Taf4; transcription initiation factor TFIID subunit 4 [KO:K03129]
685322  Uqcr10; ubiquinol-cytochrome c reductase complex 7.2kDa protein [KO:K00419]
685596  Uqcrbl1; cytochrome b-c1 complex subunit 7-like [KO:K00417]
685664  Dnal1; dynein axonemal light chain 1 [KO:K10411]
687508  Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270]
688022  Atp5pdl1; ATP synthase subunit d, mitochondrial-like [KO:K02138]
688386  Cox7a2l2; cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
688869  Cox6b1; cytochrome c oxidase subunit 6B1 [KO:K02267]
689030  Tbpl1; TATA box-binding protein-like 1 [KO:K03120]
689108  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
689688  Atg2a; autophagy-related protein 2 homolog A [KO:K17906]
689938  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
689961  Atp5mc1l1; ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
690288  Dnah14; dynein axonemal heavy chain 14 isoform X1 [KO:K10408]
690675  Cycsl2; cytochrome c, somatic-like [KO:K08738]
690848  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
690966  Polr2e; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
691001  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
78963  Apaf1; apoptotic protease-activating factor 1 [KO:K02084]
81637  Ap2a2; AP-2 complex subunit alpha-2 [KO:K11824]
81646  Creb1; cyclic AMP-responsive element-binding protein 1 isoform A [KO:K05870]
81666  Gnaq; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
81728  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial precursor [KO:K03943] [EC:7.1.1.2]
81827  Psmc5; 26S proteasome regulatory subunit 8 [KO:K03066]
83474  Tfam; transcription factor A, mitochondrial precursor [KO:K11830]
83499  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
83503  Polr2f; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
83516  Ppargc1a; peroxisome proliferator-activated receptor gamma coactivator 1-alpha [KO:K07202]
83529  Vdac1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
83531  Vdac2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
83532  Vdac3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
83618  Rest; RE1-silencing transcription factor [KO:K09222]
83800  Clta; clathrin light chain A [KO:K04644]
83806  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
84428  Dctn4; dynactin subunit 4 [KO:K10426]
84576  Hdac1l; histone deacetylase 1-like [KO:K06067] [EC:3.5.1.98]
84577  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
84683  Cox4i2; cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform 2 precursor [KO:K02263]
85240  Plcb2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
85333  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
85492  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
94194  Cox5b; cytochrome c oxidase subunit 5B, mitochondrial precursor [KO:K02265]
94198  Psmb1; proteasome subunit beta type-1 precursor [KO:K02732] [EC:3.4.25.1]
94271  Atp5pf; ATP synthase-coupling factor 6, mitochondrial precursor [KO:K02131]
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
rno00190  Oxidative phosphorylation
rno03020  RNA polymerase
rno03022  Basal transcription factors
rno03050  Proteasome
rno04020  Calcium signaling pathway
rno04115  p53 signaling pathway
rno04140  Autophagy - animal
rno04141  Protein processing in endoplasmic reticulum
rno04144  Endocytosis
rno04210  Apoptosis
rno04724  Glutamatergic synapse
KO pathway
ko05016   

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