KEGG   PATHWAY: ggo05016
Entry
ggo05016                    Pathway                                
Name
Huntington disease - Gorilla gorilla gorilla (western lowland gorilla)
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
ggo05016  Huntington disease
ggo05016

Organism
Gorilla gorilla gorilla (western lowland gorilla) [GN:ggo]
Gene
101151468  CLTA; clathrin light chain A isoform X1 [KO:K04644]
101126779  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
101153825  CLTC; clathrin heavy chain 1 isoform X2 [KO:K04646]
101132945  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
101129303  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
101150031  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
101145548  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
101146197  LOW QUALITY PROTEIN: AP-2 complex subunit beta-like [KO:K11825]
101131346  AP2S1; AP-2 complex subunit sigma isoform X1 [KO:K11827]
101125657  HTT; huntingtin isoform X1 [KO:K04533]
101143176  DCTN1; dynactin subunit 1 isoform X7 [KO:K04648]
101145272  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
101127533  DCTN3; dynactin subunit 3 [KO:K10425]
101130243  DCTN4; dynactin subunit 4 isoform X2 [KO:K10426]
101141857  DCTN5; dynactin subunit 5 isoform X1 [KO:K10427]
101151218  DCTN6; dynactin subunit 6 [KO:K10428]
101143923  ACTR10; actin-related protein 10 [KO:K16576]
101131809  HAP1; huntingtin-associated protein 1 isoform X3 [KO:K04647]
101141829  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
101152989  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
101143906  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
101149995  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
101154260  DNAH14; dynein axonemal heavy chain 14 isoform X3 [KO:K10408]
101144339  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
101149927  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
101154181  DNAH3; dynein axonemal heavy chain 3 isoform X3 [KO:K10408]
101125412  DNAH1; dynein axonemal heavy chain 1 isoform X1 [KO:K10408]
101143586  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
101136719  DNAH2; dynein axonemal heavy chain 2 isoform X1 [KO:K10408]
101132094  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
101153747  DNAI1; dynein axonemal intermediate chain 1 isoform X5 [KO:K10409]
129530372  dynein axonemal intermediate chain 1-like [KO:K10409]
101135166  DNAI2; dynein axonemal intermediate chain 2 isoform X1 [KO:K11143]
101141361  DNAL1; dynein axonemal light chain 1 [KO:K10411]
101132070  DNAL4; dynein axonemal light chain 4 [KO:K10412]
101145095  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
101149435  TUBAL3; tubulin alpha chain-like 3 isoform X1 [KO:K07374]
101136583  tubulin alpha-3 chain [KO:K07374]
101148853  tubulin alpha-4A chain [KO:K07374]
101128742  LOW QUALITY PROTEIN: tubulin alpha-1A chain [KO:K07374]
115933728  tubulin alpha-3 chain-like [KO:K07374]
101135000  TUBA3E; tubulin alpha-3E chain [KO:K07374]
129526434  tubulin alpha-3 chain [KO:K07374]
109025051  tubulin beta-8 chain isoform X1 [KO:K07375]
101135202  TUBB1; tubulin beta-1 chain isoform X2 [KO:K07375]
101125081  tubulin beta-2B chain [KO:K07375]
101134220  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
101125238  TUBB8; tubulin beta-8 chain isoform X5 [KO:K07375]
101153430  tubulin beta-8 chain-like isoform X5 [KO:K07375]
101131674  LOW QUALITY PROTEIN: melanocyte-stimulating hormone receptor [KO:K07375]
115935411  tubulin beta-8 chain isoform X1 [KO:K07375]
129523454  tubulin beta-8 chain-like isoform X4 [KO:K07375]
129534300  tubulin beta chain-like [KO:K07375]
101152026  MAPK9; mitogen-activated protein kinase 9 isoform X4 [KO:K04440] [EC:2.7.11.24]
101124033  MAPK8; mitogen-activated protein kinase 8 isoform X4 [KO:K04440] [EC:2.7.11.24]
101133141  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
101145639  KIF5A; kinesin heavy chain [KO:K10396]
101136770  KIF5B; kinesin-1 heavy chain [KO:K10396]
101130460  KIF5C; kinesin heavy chain [KO:K10396]
101142449  KLC2; kinesin light chain 2 isoform X3 [KO:K10407]
101136859  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
101146998  KLC4; kinesin light chain 4 isoform X2 [KO:K10407]
101145175  KLC1; kinesin light chain 1 isoform X30 [KO:K10407]
101132273  IFT57; intraflagellar transport protein 57 homolog [KO:K04638]
101138898  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
101126742  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
101148125  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
101149651  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
101140350  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X4 [KO:K05858] [EC:3.1.4.11]
101153613  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
101129357  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
101154323  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X2 [KO:K05858] [EC:3.1.4.11]
101123776  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
101146203  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X9 [KO:K05208]
101127573  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101151145  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
101145671  DLG4; disks large homolog 4 isoform X10 [KO:K11828]
101145558  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
101146262  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
101128020  GRIA3; glutamate receptor 3 isoform X4 [KO:K05199]
101140968  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
101154327  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
101136491  SLC1A3; excitatory amino acid transporter 1 isoform X1 [KO:K05614]
101141334  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
101130920  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
101143077  REST; RE1-silencing transcription factor [KO:K09222]
101150925  SIN3A; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
101151959  HDAC1; histone deacetylase 1 isoform X1 [KO:K06067] [EC:3.5.1.98]
109023024  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
101125439  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
101143816  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
101137939  POLR2C; DNA-directed RNA polymerase II subunit RPB3 isoform X1 [KO:K03011]
101125758  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
101153862  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X4 [KO:K03014]
101128175  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
101130252  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
101130894  DNA-directed RNA polymerase II subunit RPB11-b2-like isoform X2 [KO:K03008]
109027627  DNA-directed RNA polymerase II subunit RPB11-a isoform X1 [KO:K03008]
115933167  DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
115935376  DNA-directed RNA polymerase II subunit RPB11-A-like isoform X1 [KO:K03008]
101147508  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
109029002  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
109029365  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
101134399  BDNF; brain-derived neurotrophic factor isoform X2 [KO:K04355]
101144808  CREBBP; CREB-binding protein isoform X2 [KO:K04498] [EC:2.3.1.48]
101153383  EP300; histone acetyltransferase p300 isoform X2 [KO:K04498] [EC:2.3.1.48]
101143103  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
101151208  TAF4; transcription initiation factor TFIID subunit 4 isoform X1 [KO:K03129]
101134300  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
101125579  TBP; TATA-box-binding protein [KO:K03120]
101144410  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X3 [KO:K05870]
101128609  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
101144135  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
101144628  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X1 [KO:K09048]
101152081  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
101153965  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X4 [KO:K09047]
101133629  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 [KO:K07202]
101134077  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
101124511  TFAM; transcription factor A, mitochondrial isoform X1 [KO:K11830]
101129191  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
101127142  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
101146178  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
101151407  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
101146165  GPX5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
101134540  GPX1; glutathione peroxidase 1 isoform X1 [KO:K00432] [EC:1.11.1.9]
101138634  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
101132915  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
109023911  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
101144834  GPX6; LOW QUALITY PROTEIN: glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
101141618  UCP1; mitochondrial brown fat uncoupling protein 1 isoform X1 [KO:K08769]
101133524  TP53; cellular tumor antigen p53 isoform X2 [KO:K04451]
101152769  BAX; apoptosis regulator BAX isoform X3 [KO:K02159]
6742683  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
6742677  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
6742686  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
6742687  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
6742685  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
6742681  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
6742680  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
101142811  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
101146734  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
101131474  NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial-like [KO:K03943] [EC:7.1.1.2]
101147133  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
101144612  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
101147705  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
101124353  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101139475  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
101148914  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101152913  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949]
101135077  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
101132047  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X2 [KO:K03951]
101144771  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
101146483  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
101140479  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
101123984  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
101148753  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
101124593  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
101132014  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X1 [KO:K03957]
134758050  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
109025103  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958]
101132493  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X1 [KO:K03959]
101129506  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
101128800  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
101148993  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
101133566  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
115931649  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
101140954  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
101125598  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
101130361  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
101130165  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
101139264  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
101153768  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X2 [KO:K03935] [EC:7.1.1.2]
101133932  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
101125075  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
101146625  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
101141794  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 isoform X1 [KO:K03938]
101151434  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
101149494  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
101148614  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
101134553  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101142450  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
101141874  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
101124208  uncharacterized protein LOC101124208 isoform X1 [KO:K00234] [EC:1.3.5.1]
101131972  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101127794  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
109028866  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
101141792  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
101131106  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
6742684  CYTB; cytochrome b [KO:K00412]
101133451  cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413]
101150911  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
101130739  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
101132095  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
101132458  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
101142327  cytochrome b-c1 complex subunit 6, mitochondrial-like [KO:K00416]
101127528  cytochrome b-c1 complex subunit 7 [KO:K00417]
109027080  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101150857  cytochrome b-c1 complex subunit 7-like [KO:K00417]
101144095  cytochrome b-c1 complex subunit 8 [KO:K00418]
101123836  cytochrome b-c1 complex subunit 8-like [KO:K00418]
101132438  cytochrome b-c1 complex subunit 9 [KO:K00419]
109024713  cytochrome b-c1 complex subunit 9-like [KO:K00419]
101152996  cytochrome b-c1 complex subunit 10 [KO:K00420]
6742679  COX3; cytochrome c oxidase subunit III [KO:K02262]
6742689  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
6742682  COX2; cytochrome c oxidase subunit II [KO:K02261]
101151536  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
101141650  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X2 [KO:K02263]
101135385  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263]
101134792  COX5A; cytochrome c oxidase subunit 5A, mitochondrial isoform X1 [KO:K02264]
101138406  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
101131208  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
101133285  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
101139203  cytochrome c oxidase subunit 6B1 [KO:K02267]
101144243  cytochrome c oxidase subunit 6C [KO:K02268]
101129299  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
101144032  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
101148899  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
115934363  putative cytochrome c oxidase subunit 7A3, mitochondrial [KO:K02270]
101147253  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
101133386  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
101127312  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
101133649  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
101137162  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
109029292  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
101144277  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
134757140  cytochrome c oxidase subunit 8C, mitochondrial [KO:K02273]
101128101  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
101151032  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
101133917  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
101146631  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
109029312  ATP synthase subunit epsilon-like protein, mitochondrial [KO:K02135]
115931706  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
6742688  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
101131806  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
101133231  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
101125761  ATP5MC2; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
101144419  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
101144921  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
101138209  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
101152743  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
101141427  ATP synthase subunit d, mitochondrial-like [KO:K02138]
101145094  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
101130400  ATP5PF; ATP synthase-coupling factor 6, mitochondrial isoform X2 [KO:K02131]
6742678  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
101127243  VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862]
115935285  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
109024694  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
101142297  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
101132025  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
101143704  SLC25A31; ADP/ATP translocase 4 isoform X1 [KO:K05863]
101138376  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
129530050  ADP/ATP translocase 3 [KO:K05863]
101135431  cytochrome c [KO:K08738]
101152462  cytochrome c-like [KO:K08738]
101129170  cytochrome c-like [KO:K08738]
109025909  cytochrome c [KO:K08738]
109027916  cytochrome c-like [KO:K08738]
115932158  cytochrome c-like [KO:K08738]
101146129  cytochrome c, somatic-like [KO:K08738]
129524120  cytochrome c-like [KO:K08738]
129527250  cytochrome c-like [KO:K08738]
129526436  cytochrome c-like [KO:K08738]
129526136  cytochrome c-like [KO:K08738]
115933428  cytochrome c-like [KO:K08738]
101135722  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
101130414  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
101131305  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
129530405  proteasome subunit alpha type-6-like [KO:K02730] [EC:3.4.25.1]
101125092  PSMA2; proteasome subunit alpha type-2 isoform X1 [KO:K02726] [EC:3.4.25.1]
109024670  proteasome subunit alpha type-2-like [KO:K02726] [EC:3.4.25.1]
101137931  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
101141876  PSMA8; proteasome subunit alpha-type 8 isoform X1 [KO:K02731] [EC:3.4.25.1]
101151568  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
101151849  PSMA5; proteasome subunit alpha type-5 isoform X2 [KO:K02729] [EC:3.4.25.1]
109028113  proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
129525664  proteasome subunit alpha type-5-like isoform X1 [KO:K02729] [EC:3.4.25.1]
129525329  proteasome subunit alpha type-5-like [KO:K02729] [EC:3.4.25.1]
101147397  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
101143558  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
101136619  PSMB6; proteasome subunit beta type-6 isoform X1 [KO:K02738] [EC:3.4.25.1]
101151594  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101150208  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
101148938  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
101128203  PSMB5; proteasome subunit beta type-5 isoform X1 [KO:K02737] [EC:3.4.25.1]
101127135  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
101128397  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
101139085  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
101148521  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
101135054  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
101153204  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
101145965  26S proteasome regulatory subunit 10B-like [KO:K03064]
101128863  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 6A [KO:K03065]
101152961  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
101153440  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
101143222  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
101152227  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X2 [KO:K06693]
101126621  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
101147218  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
101151153  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 isoform X1 [KO:K03037]
101126869  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
101126459  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
101154371  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X2 [KO:K03029]
101138477  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
101153136  ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
101152290  SEM1; 26S proteasome complex subunit SEM1 isoform X1 [KO:K10881]
109026848  26S proteasome complex subunit SEM1-like [KO:K10881]
101148067  MAP3K10; mitogen-activated protein kinase kinase kinase 10 isoform X2 [KO:K04418] [EC:2.7.11.25]
101133025  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101144745  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
101147354  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
101124339  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X4 [KO:K04431] [EC:2.7.12.2]
101138990  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
101149691  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
101133270  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
101149461  ATG101; autophagy-related protein 101 [KO:K19730]
101147052  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
101135212  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
101144271  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
101130002  BECN1; beclin-1 isoform X1 [KO:K08334]
101131888  BECN2; beclin-2 [KO:K08334]
101143072  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
101139523  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
101138005  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101140067  LOW QUALITY PROTEIN: nuclear receptor-binding factor 2-like [KO:K21246]
101152991  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
101134087  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
101130514  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908]
101148284  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
101129375  TUBA1C; tubulin alpha-1C chain [KO:K07374]
101129890  TUBB; tubulin beta chain [KO:K07375]
101131473  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
101133468  ACTR1A; alpha-centractin [KO:K16575]
101137576  TUBB4A; tubulin beta-4A chain [KO:K07375]
101138770  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
101140107  RCOR1; REST corepressor 1 [KO:K11829]
101140152  uncharacterized protein LOC101140152 isoform X1 [KO:K02267]
101142053  TUBB4B; tubulin beta-4B chain [KO:K07375]
101144425  AP2M1; AP-2 complex subunit mu [KO:K11826]
101144849  STX1A; syntaxin-1A isoform X1 [KO:K04560]
101147395  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
101148105  VDAC2; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 2 [KO:K15040]
101148972  tubulin alpha-1B chain [KO:K07374]
101150168  tubulin alpha-1A chain [KO:K07374]
101153285  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
101153929  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
109024910  ACTR1B; beta-centractin isoform X1 [KO:K16575]
109025561  TUBB2A; tubulin beta-2A chain [KO:K07375]
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
ggo00190  Oxidative phosphorylation
ggo03020  RNA polymerase
ggo03022  Basal transcription factors
ggo03050  Proteasome
ggo04020  Calcium signaling pathway
ggo04115  p53 signaling pathway
ggo04140  Autophagy - animal
ggo04141  Protein processing in endoplasmic reticulum
ggo04144  Endocytosis
ggo04210  Apoptosis
ggo04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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