KEGG   PATHWAY: pps05016
Entry
pps05016                    Pathway                                
Name
Huntington disease - Pan paniscus (bonobo)
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
pps05016  Huntington disease
pps05016

Organism
Pan paniscus (bonobo) [GN:pps]
Gene
100968446  CLTA; clathrin light chain A isoform X4 [KO:K04644]
100970708  CLTB; clathrin light chain B isoform X1 [KO:K04645]
100976560  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
100980300  CLTC; clathrin heavy chain 1 isoform X2 [KO:K04646]
117978262  huntingtin-interacting protein 1 isoform X1 [KO:K04559]
100993531  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
100990247  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
100989268  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
100970095  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
100978544  LOW QUALITY PROTEIN: AP-2 complex subunit beta-like [KO:K11825]
100978720  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
100985435  AP2S1; AP-2 complex subunit sigma isoform X1 [KO:K11827]
100978882  HTT; huntingtin isoform X1 [KO:K04533]
100992136  DCTN1; dynactin subunit 1 isoform X6 [KO:K04648]
100981249  DCTN2; dynactin subunit 2 isoform X3 [KO:K10424]
100975631  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
100968332  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
100993207  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
103787109  LOW QUALITY PROTEIN: dynactin subunit 5-like [KO:K10427]
100980033  DCTN6; dynactin subunit 6 isoform X1 [KO:K10428]
100971719  ACTR1B; beta-centractin isoform X1 [KO:K16575]
100987049  ACTR1A; alpha-centractin [KO:K16575]
100979817  ACTR10; actin-related protein 10 isoform X3 [KO:K16576]
100975634  HAP1; huntingtin-associated protein 1 isoform X4 [KO:K04647]
100985094  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
100990841  DNAH9; dynein heavy chain 9, axonemal isoform X2 [KO:K10408]
100976843  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
100977574  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
100973376  DNAH10; dynein heavy chain 10, axonemal [KO:K10408]
100975690  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
100968778  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
100972652  DNAH2; dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
100986010  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
100968487  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
100973810  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
100970422  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
100972569  DNAI1; dynein intermediate chain 1, axonemal isoform X2 [KO:K10409]
100983174  DNAI2; dynein intermediate chain 2, axonemal isoform X1 [KO:K11143]
100973803  DNAL1; dynein light chain 1, axonemal isoform X1 [KO:K10411]
100981111  DNAL4; dynein light chain 4, axonemal [KO:K10412]
100995882  tubulin alpha-4A chain isoform X1 [KO:K07374]
100986068  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
100989319  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
103782837  tubulin alpha-1B chain [KO:K07374]
100993591  tubulin alpha-3 chain [KO:K07374]
100970502  TUBA1C; tubulin alpha-1C chain isoform X1 [KO:K07374]
100993058  tubulin alpha-1A chain isoform X1 [KO:K07374]
100973193  tubulin alpha-3 chain [KO:K07374]
100975223  tubulin alpha-3 chain-like [KO:K07374]
100980448  tubulin beta-3 chain isoform X3 [KO:K07375]
100971602  tubulin beta-2B chain [KO:K07375]
100978646  TUBB1; tubulin beta-1 chain [KO:K07375]
100971878  TUBB8; tubulin beta-8 chain isoform X2 [KO:K07375]
100984358  tubulin beta-1 chain-like [KO:K07375]
100991636  TUBB4A; tubulin beta-4A chain [KO:K07375]
103785843  TUBB4B; tubulin beta-4B chain [KO:K07375]
100992883  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
117974278  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
106634364  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
117978453  tubulin beta-8 chain-like isoform X1 [KO:K07375]
100970807  tubulin beta chain-like [KO:K07375]
117978671  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
100995450  LOW QUALITY PROTEIN: tubulin beta-8 chain-like [KO:K07375]
100972188  TUBB2A; tubulin beta-2A chain [KO:K07375]
100980684  TUBB; tubulin beta chain [KO:K07375]
117976737  tubulin beta 8B-like isoform X1 [KO:K07375]
100984173  MAPK10; mitogen-activated protein kinase 10 isoform X3 [KO:K04440] [EC:2.7.11.24]
100982480  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
100990080  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
100995511  KIF5B; kinesin-1 heavy chain [KO:K10396]
100976454  KIF5C; kinesin heavy chain [KO:K10396]
100978963  KIF5A; kinesin heavy chain [KO:K10396]
100974485  KLC1; kinesin light chain 1 isoform X23 [KO:K10407]
100990116  kinesin light chain 2 isoform X5 [KO:K10407]
100990383  KLC4; kinesin light chain 4 [KO:K10407]
100991175  KLC3; kinesin light chain 3 [KO:K10407]
100988293  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
100978497  CASP8; caspase-8 isoform X4 [KO:K04398] [EC:3.4.22.61]
100974934  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
100988134  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
100986730  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634]
100969660  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X2 [KO:K05858] [EC:3.1.4.11]
100987524  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
100969344  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X4 [KO:K05858] [EC:3.1.4.11]
100978643  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
100976447  STX1A; syntaxin-1A isoform X2 [KO:K04560]
100979369  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
100987673  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
100974219  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
100984884  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
100985837  DLG4; disks large homolog 4 isoform X4 [KO:K11828]
100984407  GRIA1; glutamate receptor 1 isoform X2 [KO:K05197]
100979970  GRIA2; glutamate receptor 2 isoform X2 [KO:K05198]
100995334  GRIA3; glutamate receptor 3 isoform X2 [KO:K05199]
100989781  GRIA4; glutamate receptor 4 isoform X3 [KO:K05200]
100994169  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
100977276  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
100988782  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
100977423  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
100968041  REST; RE1-silencing transcription factor [KO:K09222]
100972489  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
100969091  RCOR1; REST corepressor 1 [KO:K11829]
100980497  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
100978715  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
100978542  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
100968738  POLR2B; DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
100978061  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
100969259  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
100995503  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
100972387  DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X4 [KO:K03014]
100968084  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
100980213  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 isoform X2 [KO:K03016]
100995061  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
117974823  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
112440089  LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
117981057  DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
117980930  DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
100968278  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
112436904  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
100983866  BDNF; brain-derived neurotrophic factor isoform X3 [KO:K04355]
100983185  EP300; histone acetyltransferase p300 isoform X2 [KO:K04498] [EC:2.3.1.48]
100984888  CREBBP; CREB-binding protein isoform X3 [KO:K04498] [EC:2.3.1.48]
100972687  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
100970035  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
100977267  TBP; TATA-box-binding protein isoform X1 [KO:K03120]
100986094  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
100982500  TBPL1; TATA box-binding protein-like protein 1 isoform X1 [KO:K03120]
100985203  SP1; transcription factor Sp1 isoform X2 [KO:K04684]
100974672  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X2 [KO:K05870]
100986194  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
100981063  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
100977804  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
100989680  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
100977358  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
100973232  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X2 [KO:K09047]
100990453  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X4 [KO:K07202]
100974825  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
100974915  TFAM; transcription factor A, mitochondrial isoform X2 [KO:K11830]
100986525  NRF1; nuclear respiratory factor 1 isoform X3 [KO:K11831]
100970531  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100989293  superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
100990423  SOD2; superoxide dismutase [Mn], mitochondrial isoform X3 [KO:K04564] [EC:1.15.1.1]
100995983  GPX1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
100993851  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100984958  GPX8; probable glutathione peroxidase 8 isoform X2 [KO:K00432] [EC:1.11.1.9]
100995148  GPX5; LOW QUALITY PROTEIN: epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
100995495  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
100995142  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
100967764  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
100986239  UCP1; mitochondrial brown fat uncoupling protein 1 isoform X1 [KO:K08769]
100973941  TP53; cellular tumor antigen p53 [KO:K04451]
100991820  BAX; apoptosis regulator BAX isoform X3 [KO:K02159]
100974112  BBC3; bcl-2-binding component 3 isoform X3 [KO:K10132]
807884  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
807883  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
807879  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
807875  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
807881  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
807878  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
807873  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
100990916  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
100985717  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
100976506  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
100983914  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
100972118  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
100984764  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X2 [KO:K03947]
100967281  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
117975638  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
117976149  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
100981929  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
100989383  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112441207  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112439274  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
117977739  LOW QUALITY PROTEIN: cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
117977686  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
112437441  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
100978127  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X2 [KO:K03949]
100993915  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
100972780  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
100991958  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
100985908  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
100968569  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
100995502  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
100989931  acyl carrier protein, mitochondrial [KO:K03955]
100983552  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 isoform X2 [KO:K03956]
100991674  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
117976875  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
100977796  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
117979653  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
100972346  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958]
100976133  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100995161  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
100974158  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
100991048  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
100969637  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
100969143  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962]
100972531  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
100970265  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
100978922  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
100976767  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9-like [KO:K03965]
100968052  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
100983710  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
100967685  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
100978992  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
100996082  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
100980708  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937]
100990939  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
106633976  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
100985357  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
103785305  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
100994466  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941] [EC:7.1.1.2]
100980986  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
100975355  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
100987473  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
100989574  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
100974183  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
100975025  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
117979961  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
117979963  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
100992849  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
100974890  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
117977402  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
807882  CYTB; cytochrome b [KO:K00412]
100995886  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
100977575  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
100972888  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100984974  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
100990186  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
100973130  cytochrome b-c1 complex subunit 6, mitochondrial-like [KO:K00416]
100985683  cytochrome b-c1 complex subunit 7 [KO:K00417]
100992200  cytochrome b-c1 complex subunit 7-like [KO:K00417]
100987427  cytochrome b-c1 complex subunit 8 [KO:K00418]
100968432  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
100991862  cytochrome b-c1 complex subunit 9-like [KO:K00419]
100970584  cytochrome b-c1 complex subunit 10 [KO:K00420]
807877  COX3; cytochrome c oxidase subunit III [KO:K02262]
807872  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
807874  COX2; cytochrome c oxidase subunit II [KO:K02261]
100980404  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
100969890  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X2 [KO:K02263]
100992865  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
100988094  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
100972878  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
103783978  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
100974033  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
100975216  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
100972703  cytochrome c oxidase subunit 6B1 [KO:K02267]
100983343  cytochrome c oxidase subunit 6B2 [KO:K02267]
100994763  cytochrome c oxidase subunit 6C [KO:K02268]
100981677  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
100973061  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
117979879  putative cytochrome c oxidase subunit 7A3, mitochondrial [KO:K02270]
100968761  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
100986707  cytochrome c oxidase subunit 7B, mitochondrial isoform X2 [KO:K02271]
100982199  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
117976096  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
117977251  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
100979972  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
112436905  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
112441004  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
100984553  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
103783322  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
100968448  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
100968407  ATP5F1B; ATP synthase subunit beta, mitochondrial isoform X1 [KO:K02133] [EC:7.1.2.2]
100970277  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
100994478  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
100979899  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
117975522  ATP synthase subunit epsilon-like protein, mitochondrial [KO:K02135]
807880  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
100971737  ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100979550  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
100984833  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
100970382  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
100980736  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
117975655  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
100994408  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
100993691  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
117979430  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
100971313  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
100996147  ATP synthase subunit d, mitochondrial-like [KO:K02138]
100993404  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
100969302  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
807876  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
100983168  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
100982601  voltage-dependent anion-selective channel protein 1 [KO:K05862]
100990596  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
100987711  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X2 [KO:K15041]
100991246  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
100984012  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
100981051  ADP/ATP translocase 3-like [KO:K05863]
100971042  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
100970528  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
100986481  cytochrome c [KO:K08738]
103785895  cytochrome c [KO:K08738]
106635415  cytochrome c-like [KO:K08738]
106635123  cytochrome c-like [KO:K08738]
117975808  cytochrome c-like [KO:K08738]
117976221  cytochrome c-like [KO:K08738]
106634558  cytochrome c, somatic-like [KO:K08738]
106634335  cytochrome c-like [KO:K08738]
100969626  cytochrome c-like [KO:K08738]
117981750  cytochrome c-like [KO:K08738]
100995524  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
100992567  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
100988484  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
100972189  LOW QUALITY PROTEIN: proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
100981250  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
100990602  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
100973045  PSMA8; proteasome subunit alpha-type 8 isoform X2 [KO:K02731] [EC:3.4.25.1]
100970735  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
100972383  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
100972266  PSMA1; proteasome subunit alpha type-1 isoform X2 [KO:K02725] [EC:3.4.25.1]
100979242  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
100985958  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
100995451  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
100969694  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
100972843  proteasome subunit beta type-3-like [KO:K02735] [EC:3.4.25.1]
100995258  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
100988289  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
100977823  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
100990503  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
100987282  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
100967558  PSMC1; 26S proteasome regulatory subunit 4 isoform X1 [KO:K03062]
100970738  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
100993683  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
100968111  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
100995677  26S proteasome regulatory subunit 10B-like [KO:K03064]
100968688  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
100969119  PSMC5; 26S proteasome regulatory subunit 8 isoform X2 [KO:K03066]
100976711  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
100985251  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
100970661  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
100985092  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693]
100993559  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
100984564  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
100978445  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
100969487  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
100972978  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X2 [KO:K03039]
100995531  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
117981154  26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
100984914  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
100985395  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
100987059  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
100967271  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
100977883  MAP3K10; mitogen-activated protein kinase kinase kinase 10 isoform X1 [KO:K04418] [EC:2.7.11.25]
100975737  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100982095  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
100991014  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
100989537  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
100980204  MTOR; serine/threonine-protein kinase mTOR isoform X1 [KO:K07203] [EC:2.7.11.1]
100970765  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
100971519  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
100976889  ATG101; autophagy-related protein 101 [KO:K19730]
100978003  ATG13; autophagy-related protein 13 isoform X2 [KO:K08331]
100974589  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
100976893  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X9 [KO:K17985]
100971207  BECN1; beclin-1 isoform X1 [KO:K08334]
100994779  BECN2; beclin-2 [KO:K08334]
100968568  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 isoform X1 [KO:K08333] [EC:2.7.11.1]
100986419  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
100989794  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
100975178  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
100968213  ATG2A; autophagy-related protein 2 homolog A isoform X2 [KO:K17906]
100974220  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
100968649  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X2 [KO:K17908]
100985903  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X3 [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
pps00190  Oxidative phosphorylation
pps03020  RNA polymerase
pps03022  Basal transcription factors
pps03050  Proteasome
pps04020  Calcium signaling pathway
pps04115  p53 signaling pathway
pps04140  Autophagy - animal
pps04141  Protein processing in endoplasmic reticulum
pps04144  Endocytosis
pps04210  Apoptosis
pps04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

DBGET integrated database retrieval system