KEGG   PATHWAY: mmu05016
Entry
mmu05016                    Pathway                                
Name
Huntington disease - Mus musculus (house 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
mmu05016  Huntington disease
mmu05016

Disease
H00059  Huntington disease
Organism
Mus musculus (house mouse) [GN:mmu]
Gene
100041273  Ndufb4c; NADH:ubiquinone oxidoreductase subunit B4C [KO:K03960]
100042503  Ndufb4b; NADH:ubiquinone oxidoreductase subunit B4B [KO:K03960]
100101919  Dnah7c; dynein, axonemal, heavy chain 7C [KO:K10408]
100502727  Gm19340; predicted gene, 19340 [KO:K03949]
100504663  Atg14; autophagy related 14 [KO:K17889]
102631912  Ndufb1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
102633050  Gm6293; predicted pseudogene 6293 [KO:K00419]
102633230  Gm11633; predicted gene 11633 [KO:K02264]
102634451  Ndufab1-ps; NADH:ubiquinone oxidoreductase subunit AB1b [KO:K03955]
104130  Ndufb11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
105000  Dnal1; dynein, axonemal, light chain 1 [KO:K10411]
105675  Ppif; peptidylprolyl isomerase F (cyclophilin F) [KO:K09565] [EC:5.2.1.8]
108071  Grm5; glutamate receptor, metabotropic 5 [KO:K04604]
110082  Dnah5; dynein, axonemal, heavy chain 5 [KO:K10408]
110083  Dnah12; dynein, axonemal, heavy chain 12 [KO:K10408]
110084  Dnah1; dynein, axonemal, heavy chain 1 [KO:K10408]
110323  Cox6b1; cytochrome c oxidase, subunit 6B1 [KO:K02267]
11739  Slc25a4; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 4 [KO:K05863]
11740  Slc25a5; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 5 [KO:K05863]
11771  Ap2a1; adaptor-related protein complex 2, alpha 1 subunit [KO:K11824]
11772  Ap2a2; adaptor-related protein complex 2, alpha 2 subunit [KO:K11824]
11773  Ap2m1; adaptor-related protein complex 2, mu 1 subunit [KO:K11826]
11783  Apaf1; apoptotic peptidase activating factor 1 [KO:K02084]
11946  Atp5f1a; ATP synthase F1 subunit alpha [KO:K02132]
11947  Atp5f1b; ATP synthase F1 subunit beta [KO:K02133] [EC:7.1.2.2]
11949  Atp5f1c; ATP synthase F1 subunit gamma [KO:K02136]
11950  Atp5pb; ATP synthase peripheral stalk-membrane subunit b [KO:K02127]
11951  Atp5mc1; ATP synthase membrane subunit c locus 1 [KO:K02128]
11957  Atp5pf; ATP synthase peripheral stalk subunit F6 [KO:K02131]
12028  Bax; BCL2-associated X protein [KO:K02159]
12064  Bdnf; brain derived neurotrophic factor [KO:K04355]
12287  Cacna1b; calcium channel, voltage-dependent, N type, alpha 1B subunit [KO:K04849]
12367  Casp3; caspase 3 [KO:K02187] [EC:3.4.22.56]
12370  Casp8; caspase 8 [KO:K04398] [EC:3.4.22.61]
12371  Casp9; caspase 9 [KO:K04399] [EC:3.4.22.62]
12421  Rb1cc1; RB1-inducible coiled-coil 1 [KO:K17589]
12757  Clta; clathrin light chain A [KO:K04644]
12857  Cox4i1; cytochrome c oxidase subunit 4I1 [KO:K02263]
12858  Cox5a; cytochrome c oxidase subunit 5A [KO:K02264]
12859  Cox5b; cytochrome c oxidase subunit 5B [KO:K02265]
12861  Cox6a1; cytochrome c oxidase subunit 6A1 [KO:K02266]
12862  Cox6a2; cytochrome c oxidase subunit 6A2 [KO:K02266]
12864  Cox6c; cytochrome c oxidase subunit 6C [KO:K02268]
12865  Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270]
12866  Cox7a2; cytochrome c oxidase subunit 7A2 [KO:K02270]
12867  Cox7c; cytochrome c oxidase subunit 7C [KO:K02272]
12868  Cox8a; cytochrome c oxidase subunit 8A [KO:K02273]
12869  Cox8b; cytochrome c oxidase subunit 8B [KO:K02273]
12912  Creb1; cAMP responsive element binding protein 1 [KO:K05870]
12913  Creb3; cAMP responsive element binding protein 3 [KO:K09048]
12914  Crebbp; CREB binding protein [KO:K04498] [EC:2.3.1.48]
13063  Cycs; cytochrome c, somatic [KO:K08738]
13067  Cyct; cytochrome c, testis [KO:K08738]
13191  Dctn1; dynactin 1 [KO:K04648]
13385  Dlg4; discs large MAGUK scaffold protein 4 [KO:K11828]
13411  Dnah11; dynein, axonemal, heavy chain 11 [KO:K10408]
13417  Dnah8; dynein, axonemal, heavy chain 8 [KO:K10408]
14682  Gnaq; guanine nucleotide binding protein, alpha q polypeptide [KO:K04634]
14775  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
14776  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
14778  Gpx3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
14780  Gpx5; glutathione peroxidase 5 [KO:K00432] [EC:1.11.1.9]
14799  Gria1; glutamate receptor, ionotropic, AMPA1 (alpha 1) [KO:K05197]
14800  Gria2; glutamate receptor, ionotropic, AMPA2 (alpha 2) [KO:K05198]
14802  Gria4; glutamate receptor, ionotropic, AMPA4 (alpha 4) [KO:K05200]
14810  Grin1; glutamate receptor, ionotropic, NMDA1 (zeta 1) [KO:K05208]
14812  Grin2b; glutamate receptor, ionotropic, NMDA2B (epsilon 2) [KO:K05210]
15114  Hap1; huntingtin-associated protein 1 [KO:K04647]
15182  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
15194  Htt; huntingtin [KO:K04533]
16438  Itpr1; inositol 1,4,5-trisphosphate receptor 1 [KO:K04958]
16513  Kcnj10; potassium inwardly-rectifying channel, subfamily J, member 10 [KO:K05003]
16572  Kif5a; kinesin family member 5A [KO:K10396] [EC:5.6.1.3]
16573  Kif5b; kinesin family member 5B [KO:K10396] [EC:5.6.1.3]
16574  Kif5c; kinesin family member 5C [KO:K10396] [EC:5.6.1.3]
16593  Klc1; kinesin light chain 1 [KO:K10407]
16594  Klc2; kinesin light chain 2 [KO:K10407]
170770  Bbc3; BCL2 binding component 3 [KO:K10132]
17463  Psmd7; proteasome (prosome, macropain) 26S subunit, non-ATPase, 7 [KO:K03038]
17705  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
17706  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
17708  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
17709  COX2; cytochrome c oxidase subunit II [KO:K02261]
17710  COX3; cytochrome c oxidase subunit III [KO:K02262]
17711  CYTB; cytochrome b [KO:K00412]
17716  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
17717  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
17718  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
17719  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
17720  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
17721  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
17722  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
17749  Polr2k; polymerase (RNA) II (DNA directed) polypeptide K [KO:K03009]
17991  Ndufa2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
17992  Ndufa4; Ndufa4, mitochondrial complex associated [KO:K03948]
17993  Ndufs4; NADH:ubiquinone oxidoreductase core subunit S4 [KO:K03937]
17995  Ndufv1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2]
18181  Nrf1; nuclear respiratory factor 1 [KO:K11831]
18795  Plcb1; phospholipase C, beta 1 [KO:K05858] [EC:3.1.4.11]
18796  Plcb2; phospholipase C, beta 2 [KO:K05858] [EC:3.1.4.11]
18797  Plcb3; phospholipase C, beta 3 [KO:K05858] [EC:3.1.4.11]
18798  Plcb4; phospholipase C, beta 4 [KO:K05858] [EC:3.1.4.11]
19016  Pparg; peroxisome proliferator activated receptor gamma [KO:K08530]
19017  Ppargc1a; peroxisome proliferative activated receptor, gamma, coactivator 1 alpha [KO:K07202]
19166  Psma2; proteasome subunit alpha 2 [KO:K02726] [EC:3.4.25.1]
19167  Psma3; proteasome subunit alpha 3 [KO:K02727] [EC:3.4.25.1]
19170  Psmb1; proteasome (prosome, macropain) subunit, beta type 1 [KO:K02732] [EC:3.4.25.1]
19172  Psmb4; proteasome (prosome, macropain) subunit, beta type 4 [KO:K02736] [EC:3.4.25.1]
19173  Psmb5; proteasome (prosome, macropain) subunit, beta type 5 [KO:K02737] [EC:3.4.25.1]
19175  Psmb6; proteasome (prosome, macropain) subunit, beta type 6 [KO:K02738] [EC:3.4.25.1]
19177  Psmb7; proteasome (prosome, macropain) subunit, beta type 7 [KO:K02739] [EC:3.4.25.1]
19179  Psmc1; protease (prosome, macropain) 26S subunit, ATPase 1 [KO:K03062]
19181  Psmc2; proteasome (prosome, macropain) 26S subunit, ATPase 2 [KO:K03061]
19182  Psmc3; proteasome (prosome, macropain) 26S subunit, ATPase 3 [KO:K03065]
19184  Psmc5; protease (prosome, macropain) 26S subunit, ATPase 5 [KO:K03066]
19185  Psmd4; proteasome (prosome, macropain) 26S subunit, non-ATPase, 4 [KO:K03029]
19712  Rest; RE1-silencing transcription factor [KO:K09222]
20020  Polr2a; polymerase (RNA) II (DNA directed) polypeptide A [KO:K03006] [EC:2.7.7.6]
20021  Polr2c; polymerase (RNA) II (DNA directed) polypeptide C [KO:K03011]
20022  Polr2j; polymerase (RNA) II (DNA directed) polypeptide J [KO:K03008]
20422  Sem1; SEM1, 26S proteasome complex subunit [KO:K10881]
20463  Cox7a2l; cytochrome c oxidase subunit 7A2 like [KO:K02270]
20466  Sin3a; transcriptional regulator, SIN3A (yeast) [KO:K11644]
20511  Slc1a2; solute carrier family 1 (glial high affinity glutamate transporter), member 2 [KO:K05613]
20512  Slc1a3; solute carrier family 1 (glial high affinity glutamate transporter), member 3 [KO:K05614]
20655  Sod1; superoxide dismutase 1, soluble [KO:K04565] [EC:1.15.1.1]
20656  Sod2; superoxide dismutase 2, mitochondrial [KO:K04564] [EC:1.15.1.1]
20683  Sp1; trans-acting transcription factor 1 [KO:K04684]
208647  Creb3l2; cAMP responsive element binding protein 3-like 2 [KO:K09048]
208677  Creb3l3; cAMP responsive element binding protein 3-like 3 [KO:K09048]
20907  Stx1a; syntaxin 1A (brain) [KO:K04560]
21374  Tbp; TATA box binding protein [KO:K03120]
215114  Hip1; huntingtin interacting protein 1 [KO:K04559]
21762  Psmd2; proteasome (prosome, macropain) 26S subunit, non-ATPase, 2 [KO:K03028]
21780  Tfam; transcription factor A, mitochondrial [KO:K11830]
217864  Rcor1; REST corepressor 1 [KO:K11829]
21817  Tgm2; transglutaminase 2, C polypeptide [KO:K05625] [EC:2.3.2.13]
22030  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
22059  Trp53; transformation related protein 53 [KO:K04451]
22123  Psmd3; proteasome (prosome, macropain) 26S subunit, non-ATPase, 3 [KO:K03033]
22142  Tuba1a; tubulin, alpha 1A [KO:K07374]
22143  Tuba1b; tubulin, alpha 1B [KO:K07374]
22144  Tuba3a; tubulin, alpha 3A [KO:K07374]
22145  Tuba4a; tubulin, alpha 4A [KO:K07374]
22146  Tuba1c; tubulin, alpha 1C [KO:K07374]
22147  Tuba3b; tubulin, alpha 3B [KO:K07374]
22151  Tubb2a; tubulin, beta 2A class IIA [KO:K07375]
22152  Tubb3; tubulin, beta 3 class III [KO:K07375]
22153  Tubb4a; tubulin, beta 4A class IVA [KO:K07375]
22154  Tubb5; tubulin, beta 5 class I [KO:K07375]
22227  Ucp1; uncoupling protein 1 (mitochondrial, proton carrier) [KO:K08769]
22241  Ulk1; unc-51 like kinase 1 [KO:K21357] [EC:2.7.11.1]
22272  Uqcrq; ubiquinol-cytochrome c reductase, complex III subunit VII [KO:K00418]
22273  Uqcrc1; ubiquinol-cytochrome c reductase core protein 1 [KO:K00414]
22333  Vdac1; voltage-dependent anion channel 1 [KO:K05862]
22334  Vdac2; voltage-dependent anion channel 2 [KO:K15040]
22335  Vdac3; voltage-dependent anion channel 3 [KO:K15041]
22428  Dctn6; dynactin 6 [KO:K10428]
225326  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
225887  Ndufs8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2]
226646  Ndufs2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2]
226977  Actr1b; ARP1 actin-related protein 1B, centractin beta [KO:K16575]
227058  Dnah7b; dynein, axonemal, heavy chain 7B [KO:K10408]
227197  Ndufs1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2]
227606  Tbpl2; TATA box binding protein like 2 [KO:K03120]
227613  Tubb4b; tubulin, beta 4B class IVB [KO:K07375]
228033  Atp5mc3; ATP synthase membrane subunit c locus 3 [KO:K02128]
228361  Ambra1; autophagy/beclin 1 regulator 1 [KO:K17985]
228980  Taf4; TATA-box binding protein associated factor 4 [KO:K03129]
230075  Ndufb6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
231329  Polr2b; polymerase (RNA) II (DNA directed) polypeptide B [KO:K03010] [EC:2.7.7.6]
231991  Creb5; cAMP responsive element binding protein 5 [KO:K09047]
232910  Ap2s1; adaptor-related protein complex 2, sigma 1 subunit [KO:K11827]
232943  Klc3; kinesin light chain 3 [KO:K10407]
237336  Tbpl1; TATA box binding protein-like 1 [KO:K03120]
237806  Dnah9; dynein, axonemal, heavy chain 9 [KO:K10408]
238463  Tubal3; tubulin, alpha-like 3 [KO:K07374]
23996  Psmc4; proteasome (prosome, macropain) 26S subunit, ATPase, 4 [KO:K03063]
23997  Psmd13; proteasome (prosome, macropain) 26S subunit, non-ATPase, 13 [KO:K03039]
240960  Dnah14; dynein, axonemal, heavy chain 14 [KO:K10408]
245841  Polr2h; polymerase (RNA) II (DNA directed) polypeptide H [KO:K03016]
26400  Map2k7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
26408  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
26414  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
26419  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
26420  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
26427  Creb3l1; cAMP responsive element binding protein 3-like 1 [KO:K09048]
26440  Psma1; proteasome subunit alpha 1 [KO:K02725] [EC:3.4.25.1]
26441  Psma4; proteasome subunit alpha 4 [KO:K02728] [EC:3.4.25.1]
26442  Psma5; proteasome subunit alpha 5 [KO:K02729] [EC:3.4.25.1]
26443  Psma6; proteasome subunit alpha 6 [KO:K02730] [EC:3.4.25.1]
26444  Psma7; proteasome subunit alpha 7 [KO:K02731] [EC:3.4.25.1]
26445  Psmb2; proteasome (prosome, macropain) subunit, beta type 2 [KO:K02734] [EC:3.4.25.1]
26446  Psmb3; proteasome (prosome, macropain) subunit, beta type 3 [KO:K02735] [EC:3.4.25.1]
269881  Map3k10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
28080  Atp5po; ATP synthase peripheral stalk subunit OSCP [KO:K02137]
29869  Ulk2; unc-51 like kinase 2 [KO:K08269] [EC:2.7.11.1]
327954  Dnah2; dynein, axonemal, heavy chain 2 [KO:K10408]
328572  Ep300; E1A binding protein p300 [KO:K04498] [EC:2.3.1.48]
329015  Atg2a; autophagy related 2A [KO:K17906]
330355  Dnah6; dynein, axonemal, heavy chain 6 [KO:K10408]
333182  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
381917  Dnah3; dynein, axonemal, heavy chain 3 [KO:K10408]
407785  Ndufs6; NADH:ubiquinone oxidoreductase core subunit S6 [KO:K03939]
407790  Ndufa4l2; Ndufa4, mitochondrial complex associated like 2 [KO:K03948]
432611  Dnai2; dynein axonemal intermediate chain 2 [KO:K11143]
433759  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
51897  Atg13; autophagy related 13 [KO:K08331]
52639  Wipi1; WD repeat domain, phosphoinositide interacting 1 [KO:K17908]
53598  Dctn3; dynactin 3 [KO:K10425]
53623  Gria3; glutamate receptor, ionotropic, AMPA3 (alpha 3) [KO:K05199]
53857  Tuba8; tubulin, alpha 8 [KO:K07374]
54130  Actr1a; ARP1 actin-related protein 1A, centractin alpha [KO:K16575]
54152  Dnal4; dynein, axonemal, light chain 4 [KO:K10412]
54405  Ndufa1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
545486  Tubb1; tubulin, beta 1 class VI [KO:K07375]
56087  Dnah10; dynein, axonemal, heavy chain 10 [KO:K10408]
56208  Becn1; beclin 1, autophagy related [KO:K08334]
56436  Adrm1; adhesion regulating molecule 1 26S proteasome ubiquitin receptor [KO:K06691]
56444  Actr10; ARP10 actin-related protein 10 [KO:K16576]
56717  Mtor; mechanistic target of rapamycin kinase [KO:K07203] [EC:2.7.11.1]
57296  Psmd8; proteasome (prosome, macropain) 26S subunit, non-ATPase, 8 [KO:K03031]
59029  Psmd14; proteasome (prosome, macropain) 26S subunit, non-ATPase, 14 [KO:K03030]
59288  Dctn5; dynactin 5 [KO:K10427]
595136  Ndufs5; NADH:ubiquinone oxidoreductase core subunit S5 [KO:K03938]
623286  Ndufs6b; NADH:ubiquinone oxidoreductase subunit S6B [KO:K03939]
627872  Dnah7a; dynein, axonemal, heavy chain 7A [KO:K10408]
641340  Nrbf2; nuclear receptor binding factor 2 [KO:K21246]
66043  Atp5f1d; ATP synthase F1 subunit delta [KO:K02134]
66046  Ndufb5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
66052  Sdhc; succinate dehydrogenase complex, subunit C, integral membrane protein [KO:K00236]
66091  Ndufa3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
66108  Ndufa9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
66142  Cox7b; cytochrome c oxidase subunit 7B [KO:K02271]
66152  Uqcr10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
66218  Ndufb9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
66377  Ndufc1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
66413  Psmd6; proteasome (prosome, macropain) 26S subunit, non-ATPase, 6 [KO:K03037]
66414  Ndufa12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
66416  Ndufa7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
66420  Polr2e; polymerase (RNA) II (DNA directed) polypeptide E [KO:K03013]
66445  Cyc1; cytochrome c-1 [KO:K00413]
66491  Polr2l; polymerase (RNA) II (DNA directed) polypeptide L [KO:K03007]
66495  Ndufb3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
66576  Uqcrh; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
66594  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
66694  Uqcrfs1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:7.1.1.8]
66916  Ndufb7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
66925  Sdhd; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237]
66945  Sdha; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234] [EC:1.3.5.1]
66997  Psmd12; proteasome (prosome, macropain) 26S subunit, non-ATPase, 12 [KO:K03035]
67003  Uqcrc2; ubiquinol cytochrome c reductase core protein 2 [KO:K00415]
67089  Psmc6; proteasome (prosome, macropain) 26S subunit, ATPase, 6 [KO:K03064]
67126  Atp5f1e; ATP synthase F1 subunit epsilon [KO:K02135]
67130  Ndufa6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
67151  Psmd9; proteasome (prosome, macropain) 26S subunit, non-ATPase, 9 [KO:K06693]
67184  Ndufa13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
67264  Ndufb8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
67273  Ndufa10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
67300  Cltc; clathrin heavy chain [KO:K04646]
67305  Gpx7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
67530  Uqcrb; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
67665  Dctn4; dynactin 4 [KO:K10426]
67680  Sdhb; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235] [EC:1.3.5.1]
67710  Polr2g; polymerase (RNA) II (DNA directed) polypeptide G [KO:K03015]
67942  Atp5mc2; ATP synthase membrane subunit c locus 2 [KO:K02128]
67951  Tubb6; tubulin, beta 6 class V [KO:K07375]
68118  Atg101; autophagy related 101 [KO:K19730]
68194  Ndufb4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
68197  Ndufc2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
68198  Ndufb2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
68202  Ndufa5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
68342  Ndufb10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
68349  Ndufs3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2]
68375  Ndufa8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
68922  Dnai1; dynein axonemal intermediate chain 1 [KO:K10409]
69077  Psmd11; proteasome (prosome, macropain) 26S subunit, non-ATPase, 11 [KO:K03036]
69241  Polr2d; polymerase (RNA) II (DNA directed) polypeptide D [KO:K03012]
69590  Gpx8; glutathione peroxidase 8 (putative) [KO:K00432] [EC:1.11.1.9]
69654  Dctn2; dynactin 2 [KO:K10424]
69833  Polr2f; polymerase (RNA) II (DNA directed) polypeptide F [KO:K03014]
69875  Ndufa11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
69920  Polr2i; polymerase (RNA) II (DNA directed) polypeptide I [KO:K03017]
69926  Dnah17; dynein, axonemal, heavy chain 17 [KO:K10408]
70247  Psmd1; proteasome (prosome, macropain) 26S subunit, non-ATPase, 1 [KO:K03032]
70316  Ndufab1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
71679  Atp5pd; ATP synthase peripheral stalk subunit d [KO:K02138]
71770  Ap2b1; adaptor-related protein complex 2, beta 1 subunit [KO:K11825]
72504  Taf4b; TATA-box binding protein associated factor 4b [KO:K03129]
72900  Ndufv2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2]
73333  Slc25a31; solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31 [KO:K05863]
73677  Psma8; proteasome subunit alpha 8 [KO:K02731] [EC:3.4.25.1]
73710  Tubb2b; tubulin, beta 2B class IIB [KO:K07375]
73916  Ift57; intraflagellar transport 57 [KO:K04638]
74325  Cltb; clathrin light chain B [KO:K04645]
74764  Klc4; kinesin light chain 4 [KO:K10407]
74781  Wipi2; WD repeat domain, phosphoinositide interacting 2 [KO:K17908]
75406  Ndufs7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2]
75483  Cox8c; cytochrome c oxidase subunit 8C [KO:K02273]
75512  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
75563  Dnali1; dynein, axonemal, light intermediate polypeptide 1 [KO:K10410]
75669  Pik3r4; phosphoinositide-3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
76559  Atg2b; autophagy related 2B [KO:K17906]
78174  Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271]
78284  Creb3l4; cAMP responsive element binding protein 3-like 4 [KO:K09048]
78330  Ndufv3; NADH:ubiquinone oxidoreductase core subunit V3 [KO:K03944]
78943  Ern1; endoplasmic reticulum to nucleus signalling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
84682  Cox4i2; cytochrome c oxidase subunit 4I2 [KO:K02263]
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
mmu00190  Oxidative phosphorylation
mmu03020  RNA polymerase
mmu03022  Basal transcription factors
mmu03050  Proteasome
mmu04020  Calcium signaling pathway
mmu04115  p53 signaling pathway
mmu04140  Autophagy - animal
mmu04141  Protein processing in endoplasmic reticulum
mmu04144  Endocytosis
mmu04210  Apoptosis
mmu04724  Glutamatergic synapse
KO pathway
ko05016   

  All links  
No link information was found.
DBGET integrated database retrieval system