KEGG   PATHWAY: hmh05016
Entry
hmh05016                    Pathway                                
Name
Huntington disease - Hylobates moloch (silvery gibbon)
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
hmh05016  Huntington disease
hmh05016

Organism
Hylobates moloch (silvery gibbon) [GN:hmh]
Gene
116475086  CLTA; clathrin light chain A isoform X1 [KO:K04644]
116471914  CLTB; clathrin light chain B isoform X1 [KO:K04645]
116458692  CLTCL1; clathrin heavy chain 2 isoform X3 [KO:K04646]
116474318  CLTC; clathrin heavy chain 1 [KO:K04646]
116811348  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
116478178  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
116463885  AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
116463889  AP-2 complex subunit alpha-2-like isoform X1 [KO:K11824]
116471978  LOW QUALITY PROTEIN: AP-2 complex subunit beta-like [KO:K11825]
116811353  LOW QUALITY PROTEIN: AP-2 complex subunit beta-like [KO:K11825]
116474498  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
116479892  AP2M1; AP-2 complex subunit mu [KO:K11826]
116478300  AP-2 complex subunit sigma isoform X2 [KO:K11827]
116457078  HTT; huntingtin isoform X2 [KO:K04533]
116483874  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
116480864  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
116475034  DCTN3; dynactin subunit 3 [KO:K10425]
116471723  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
116471143  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
116472309  dynactin subunit 5-like [KO:K10427]
116464853  dynactin subunit 6-like [KO:K10428]
116462135  DCTN6; dynactin subunit 6 [KO:K10428]
116484283  ACTR1A; alpha-centractin isoform X1 [KO:K16575]
116482984  ACTR1B; beta-centractin isoform X1 [KO:K16575]
116484777  ACTR10; actin-related protein 10 [KO:K16576]
116474837  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
116459055  DNAH2; dynein axonemal heavy chain 2 isoform X1 [KO:K10408]
116477715  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
116459472  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
116469188  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
116810514  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
116460257  DNAH8; dynein axonemal heavy chain 8 isoform X1 [KO:K10408]
116471111  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
116813230  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
116473403  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
116483345  DNAH6; dynein axonemal heavy chain 6 isoform X1 [KO:K10408]
116457106  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
116483747  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
116472963  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
116475381  DNAI1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
116483072  DNAI2; dynein axonemal intermediate chain 2 isoform X1 [KO:K11143]
116456326  DNAL1; dynein axonemal light chain 1 [KO:K10411]
116459401  DNAL4; dynein axonemal light chain 4 [KO:K10412]
116459291  tubulin alpha-8 chain [KO:K07374]
116468915  tubulin alpha-4A chain isoform X2 [KO:K07374]
116461664  TUBA1A; tubulin alpha-1A chain [KO:K07374]
116461665  tubulin alpha-1C chain [KO:K07374]
116461669  tubulin alpha-1B chain [KO:K07374]
116462964  tubulin alpha-3 chain [KO:K07374]
116474604  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
116467188  tubulin alpha-3 chain-like [KO:K07374]
116475928  TUBB4B; tubulin beta-4B chain [KO:K07375]
116476254  TUBB8B; LOW QUALITY PROTEIN: tubulin beta 8B [KO:K07375]
116468766  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
116460085  TUBB; tubulin beta chain [KO:K07375]
116478333  TUBB4A; tubulin beta-4A chain [KO:K07375]
116460404  tubulin beta-1 chain-like [KO:K07375]
116470666  tubulin beta-8 chain isoform X2 [KO:K07375]
116463286  tubulin beta-1 chain-like [KO:K07375]
116463372  tubulin beta-1 chain [KO:K07375]
116464297  tubulin beta-3 chain [KO:K07375]
116464528  tubulin beta-8 chain-like isoform X1 [KO:K07375]
116464579  tubulin beta-8 chain-like isoform X1 [KO:K07375]
116465307  tubulin beta-8 chain-like [KO:K07375]
116465541  tubulin beta-8 chain-like isoform X2 [KO:K07375]
116457218  tubulin beta-8 chain-like [KO:K07375]
116460769  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
116471970  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
116463128  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
116461914  KIF5B; kinesin-1 heavy chain isoform X1 [KO:K10396]
116480862  KIF5A; kinesin heavy chain isoform X1 [KO:K10396]
116462985  KIF5C; kinesin heavy chain [KO:K10396]
116469037  KLC2; kinesin light chain 2 isoform X2 [KO:K10407]
116460468  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
116456097  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
116483357  KLC3; kinesin light chain 3 [KO:K10407]
116466977  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
116810454  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
116466067  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
116476794  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
116475178  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
116468938  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
116461202  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
116461370  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X2 [KO:K05858] [EC:3.1.4.11]
116481969  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X6 [KO:K05858] [EC:3.1.4.11]
116811658  STX1A; syntaxin-1A isoform X1 [KO:K04560]
116475947  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
116475911  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X2 [KO:K05208]
116472713  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
116466681  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
116459315  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
116471746  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
116463465  glutamate receptor 2 isoform X1 [KO:K05198]
116463489  glutamate receptor 2 isoform X1 [KO:K05198]
116812014  GRIA3; glutamate receptor 3 isoform X2 [KO:K05199]
116476877  GRIA4; glutamate receptor 4 isoform X4 [KO:K05200]
116461015  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
116456906  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
116476357  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
116469219  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
116467953  REST; RE1-silencing transcription factor [KO:K09222]
116482404  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
116484816  RCOR1; REST corepressor 1 isoform X2 [KO:K11829]
116471149  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
116472879  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
116459477  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
116476581  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
116471303  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
116462957  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
116464931  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
116459400  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
116468902  POLR2G; DNA-directed RNA polymerase II subunit RPB7 isoform X1 [KO:K03015]
116479907  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X1 [KO:K03016]
116478942  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
116466605  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
116811000  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
116467257  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
116462054  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
116813469  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 isoform X2 [KO:K03009]
116476405  BDNF; brain-derived neurotrophic factor isoform X1 [KO:K04355]
116458301  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
116462715  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
116468653  TAF4B; transcription initiation factor TFIID subunit 4B isoform X2 [KO:K03129]
116461462  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
116485087  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
116472573  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
116474201  TBP; TATA-box-binding protein [KO:K03120]
116481003  SP1; transcription factor Sp1 isoform X1 [KO:K04684]
116810385  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X3 [KO:K05870]
116476303  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
116469376  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
116814406  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
116483538  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
116475074  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
116812715  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X5 [KO:K09047]
116456631  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
116466751  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
116458192  TFAM; transcription factor A, mitochondrial [KO:K11830]
116814351  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
116482392  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
116473534  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
116457764  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
116484933  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
116459940  GPX5; epididymal secretory glutathione peroxidase isoform X3 [KO:K00432] [EC:1.11.1.9]
116459941  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
116471728  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
116462435  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
116473477  GPX1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
116464311  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
116459052  TP53; cellular tumor antigen p53 [KO:K04451]
116478225  BAX; apoptosis regulator BAX isoform X3 [KO:K02159]
116478291  BBC3; bcl-2-binding component 3 isoform X1 [KO:K10132]
116469089  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942] [EC:7.1.1.2]
116468740  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X2 [KO:K03943] [EC:7.1.1.2]
116481681  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
116811998  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
116471600  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
116812249  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946]
116457665  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
116471190  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
116479184  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
116469688  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
116469796  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
116811178  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
116813617  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
116468192  cytochrome c oxidase subunit NDUFA4 [KO:K03948]
116459108  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X2 [KO:K03949]
116466356  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
116471218  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
116814302  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949]
116458807  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
116478399  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
116475617  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
116472560  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
116814514  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X3 [KO:K03954]
116471141  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
116464026  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
116464030  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
116479108  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
116478746  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 isoform X1 [KO:K11353]
116476238  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
116484857  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X1 [KO:K03957]
116814430  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
116475658  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
116456270  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
116483082  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
116811503  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
116466891  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
116481268  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
116458229  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
116478601  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
116485009  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
116813580  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
116462643  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 isoform X1 [KO:K03966]
116476180  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
116811635  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
116810400  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
116473340  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
116476267  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
116462285  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
116479923  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
116472984  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
116474589  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
116464274  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
116464279  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
116456788  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
116484171  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X2 [KO:K03940] [EC:7.1.1.2]
116469098  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
116464170  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
116476737  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
116456796  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
116812984  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
116464049  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
116473332  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
116476941  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
116814392  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like isoform X1 [KO:K00237]
116478821  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
116813689  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
116473447  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
116471120  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
116480828  cytochrome b-c1 complex subunit 6, mitochondrial-like [KO:K00416]
116473111  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
116457103  cytochrome b-c1 complex subunit 7 [KO:K00417]
116471795  cytochrome b-c1 complex subunit 7-like [KO:K00417]
116813443  cytochrome b-c1 complex subunit 7 [KO:K00417]
116480043  cytochrome b-c1 complex subunit 8-like [KO:K00418]
116470828  cytochrome b-c1 complex subunit 8 [KO:K00418]
116459021  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
116811705  cytochrome b-c1 complex subunit 9-like [KO:K00419]
116484013  cytochrome b-c1 complex subunit 10 [KO:K00420]
116484546  cytochrome b-c1 complex subunit 10-like [KO:K00420]
116478869  cytochrome b-c1 complex subunit 10-like [KO:K00420]
116470741  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
116461312  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
116467991  cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264]
116481919  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
116456926  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
116466323  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
116482986  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
116477827  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
116471196  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
116478914  cytochrome c oxidase subunit 6B1 [KO:K02267]
116479218  cytochrome c oxidase subunit 6B2 [KO:K02267]
116813467  cytochrome c oxidase subunit 6C [KO:K02268]
116468858  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
116478944  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
116483639  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
116458524  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
116460740  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
116811106  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
116812505  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
116812582  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
116456710  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
116484067  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116457035  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116457630  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116466514  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
116471025  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
116485090  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
116468931  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
116468579  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
116480897  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
116468460  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
116483820  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
116463284  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
116463374  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
116466389  ATP synthase subunit epsilon, mitochondrial [KO:K02135]
116810171  LOW QUALITY PROTEIN: ATP synthase subunit a-like [KO:K02126]
116810507  LOW QUALITY PROTEIN: ATP synthase subunit a-like [KO:K02126]
116809726  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit B1, mitochondrial-like [KO:K02127]
116469616  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
116467551  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
116468490  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
116810638  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
116811267  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
116480990  ATP5MC2; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
116479340  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
116474681  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
116483666  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
116482310  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
116482164  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
116811837  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
116471501  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
116459956  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
116461833  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
116811486  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
116811994  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
116463829  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
116466073  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
116460298  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
116476907  cytochrome c-like [KO:K08738]
116809676  cytochrome c-like [KO:K08738]
116811446  cytochrome c-like [KO:K08738]
116471160  cytochrome c [KO:K08738]
116812987  cytochrome c isoform X2 [KO:K08738]
116479141  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
116812958  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
116484618  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
116476791  LOW QUALITY PROTEIN: proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
116812514  PSMA2; proteasome subunit alpha type-2 isoform X1 [KO:K02726] [EC:3.4.25.1]
116473013  proteasome subunit alpha type-4-like [KO:K02728] [EC:3.4.25.1]
116482501  PSMA4; proteasome subunit alpha type-4 isoform X2 [KO:K02728] [EC:3.4.25.1]
116468102  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
116461471  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
116469659  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
116476488  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
116484774  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
116459066  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
116475643  PSMB7; proteasome subunit beta type-7 isoform X4 [KO:K02739] [EC:3.4.25.1]
116810323  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
116474730  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
116472932  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
116484906  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
116474191  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
116469443  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
116461712  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 7 [KO:K03061]
116814198  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
116809684  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
116471161  26S proteasome regulatory subunit 4-like [KO:K03062]
116456295  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
116480690  PSMC4; 26S proteasome regulatory subunit 6B isoform X1 [KO:K03063]
116813279  26S proteasome regulatory subunit 10B-like [KO:K03064]
116456332  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
116476272  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
116458738  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
116479902  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
116810091  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
116474767  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
116477784  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
116483230  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
116474472  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
116466621  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
116471451  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
116466559  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
116469451  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
116810748  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
116480744  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
116461465  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
116813839  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
116480038  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
116464513  mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
116464517  mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
116458727  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
116475877  TRAF2; TNF receptor-associated factor 2 isoform X3 [KO:K03173] [EC:2.3.2.27]
116472781  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
116478375  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
116812918  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
116477673  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
116483081  ULK2; serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
116461657  ATG101; autophagy-related protein 101 [KO:K19730]
116476294  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
116813298  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
116476298  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
116474889  BECN1; beclin-1 isoform X1 [KO:K08334]
116457644  BECN2; beclin-2 [KO:K08334]
116461710  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
116484834  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
116458510  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
116468590  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
116484631  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
116468972  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
116810832  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
116811370  WD repeat domain phosphoinositide-interacting protein 2-like [KO:K17908]
116483631  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
116475268  LOW QUALITY PROTEIN: WD repeat domain phosphoinositide-interacting protein 2 [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
hmh00190  Oxidative phosphorylation
hmh03020  RNA polymerase
hmh03022  Basal transcription factors
hmh03050  Proteasome
hmh04020  Calcium signaling pathway
hmh04115  p53 signaling pathway
hmh04140  Autophagy - animal
hmh04141  Protein processing in endoplasmic reticulum
hmh04144  Endocytosis
hmh04210  Apoptosis
hmh04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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