KEGG   PATHWAY: umr05016
Entry
umr05016                    Pathway                                
Name
Huntington disease - Ursus maritimus (polar bear)
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
umr05016  Huntington disease
umr05016

Organism
Ursus maritimus (polar bear) [GN:umr]
Gene
103678470  CLTA; clathrin light chain A isoform X1 [KO:K04644]
103664765  CLTB; clathrin light chain B [KO:K04645]
103667334  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
103669305  CLTCL1; clathrin heavy chain 2 [KO:K04646]
103670216  HIP1; huntingtin-interacting protein 1 [KO:K04559]
103657261  AP2A1; AP-2 complex subunit alpha-1 [KO:K11824]
103671046  AP2A2; AP-2 complex subunit alpha-2 isoform X10 [KO:K11824]
103667291  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
103676085  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
103657073  AP2S1; AP-2 complex subunit sigma [KO:K11827]
103677033  HTT; huntingtin isoform X1 [KO:K04533]
103671404  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
103656621  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
103667053  DCTN3; dynactin subunit 3 [KO:K10425]
103664591  DCTN4; dynactin subunit 4 [KO:K10426]
103668855  DCTN5; dynactin subunit 5 [KO:K10427]
103659089  DCTN6; dynactin subunit 6 [KO:K10428]
103674657  LOW QUALITY PROTEIN: dynactin subunit 6-like [KO:K10428]
103680932  ACTR1A; alpha-centractin [KO:K16575]
103671190  ACTR1B; beta-centractin isoform X1 [KO:K16575]
103673355  ACTR10; actin-related protein 10 [KO:K16576]
103672609  HAP1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
103662236  DNAH5; dynein heavy chain 5, axonemal isoform X1 [KO:K10408]
103669094  DNAH14; dynein heavy chain 14, axonemal isoform X1 [KO:K10408]
103660479  DNAH2; dynein heavy chain 2, axonemal [KO:K10408]
103660509  DNAH9; dynein heavy chain 9, axonemal [KO:K10408]
103658865  DNAH11; dynein heavy chain 11, axonemal [KO:K10408]
103659938  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
103661249  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
103680884  DNAH8; dynein heavy chain 8, axonemal [KO:K10408]
103671447  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
103668889  DNAH3; dynein heavy chain 3, axonemal [KO:K10408]
103665425  dynein heavy chain 8, axonemal-like [KO:K10408]
103665969  DNAH17; dynein heavy chain 17, axonemal [KO:K10408]
103675059  DNAH12; dynein heavy chain 12, axonemal [KO:K10408]
103675071  DNAH1; dynein heavy chain 1, axonemal [KO:K10408]
103666373  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
103667109  DNAI1; dynein intermediate chain 1, axonemal [KO:K10409]
103665919  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
103671342  DNAL1; dynein light chain 1, axonemal [KO:K10411]
103676378  DNAL4; dynein light chain 4, axonemal isoform X1 [KO:K10412]
103657723  tubulin alpha-8 chain [KO:K07374]
103662744  tubulin alpha-4A chain [KO:K07374]
103669231  tubulin alpha-3 chain isoform X1 [KO:K07374]
103669237  tubulin alpha-3 chain-like [KO:K07374]
103676280  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
103675456  TUBA1B; tubulin alpha-1B chain [KO:K07374]
103675457  tubulin alpha-1C chain [KO:K07374]
103675459  tubulin alpha-1B chain [KO:K07374]
103675693  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
103681710  TUBB4A; tubulin beta-4A chain [KO:K07375]
103673863  tubulin beta-3 chain [KO:K07375]
103673361  TUBB6; tubulin beta-6 chain [KO:K07375]
103675253  TUBB4B; tubulin beta-4B chain [KO:K07375]
103670962  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
103665004  TUBB; tubulin beta chain [KO:K07375]
103664961  TUBB2A; tubulin beta-2A chain isoform X1 [KO:K07375]
103676947  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
103679990  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
103667893  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
103656636  KIF5A; kinesin heavy chain [KO:K10396]
103656532  KIF5B; kinesin-1 heavy chain [KO:K10396]
103659841  KIF5C; kinesin heavy chain [KO:K10396]
103657183  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
103664073  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
103679486  KLC2; kinesin light chain 2 [KO:K10407]
103676885  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
103677374  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
103659742  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
103680991  CASP3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
103675379  GRM5; LOW QUALITY PROTEIN: metabotropic glutamate receptor 5 [KO:K04604]
103663281  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
103673552  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 [KO:K05858] [EC:3.1.4.11]
103674425  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
103674426  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 [KO:K05858] [EC:3.1.4.11]
103679549  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
121099746  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
103670156  STX1A; syntaxin-1A [KO:K04560]
103675324  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
103675310  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
103677769  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
103669597  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
103660461  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
103664566  GRIA1; glutamate receptor 1 isoform X2 [KO:K05197]
103658055  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
103680225  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
103662439  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
103669768  TGM2; protein-glutamine gamma-glutamyltransferase 2 isoform X1 [KO:K05625] [EC:2.3.2.13]
103664705  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
103659316  SLC1A2; excitatory amino acid transporter 2 [KO:K05613]
103672051  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
103672754  REST; RE1-silencing transcription factor [KO:K09222]
103678978  SIN3A; paired amphipathic helix protein Sin3a [KO:K11644]
103656780  RCOR1; REST corepressor 1 [KO:K11829]
103676974  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
103666443  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
103660476  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
103672816  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
103673892  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
103658564  POLR2D; DNA-directed RNA polymerase II subunit RPB4 [KO:K03012]
103681776  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
103676392  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
103678202  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
103676079  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
103682534  POLR2I; DNA-directed RNA polymerase II subunit RPB9 isoform X1 [KO:K03017]
103671102  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
103670139  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
103661503  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 isoform X1 [KO:K03009]
103659367  BDNF; brain-derived neurotrophic factor [KO:K04355]
103668810  CREB-binding protein [KO:K04498] [EC:2.3.1.48]
103674159  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
103664911  TAF4B; transcription initiation factor TFIID subunit 4B [KO:K03129]
103660585  TAF4; transcription initiation factor TFIID subunit 4 [KO:K03129]
103673156  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
103660778  TATA-box-binding protein [KO:K03120]
103663386  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
103675530  SP1; transcription factor Sp1 [KO:K04684]
103659802  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
103681679  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
103668409  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
103667087  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
103669052  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
103672393  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
103658932  cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
103658371  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha [KO:K07202]
103669544  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
103667732  TFAM; transcription factor A, mitochondrial [KO:K11830]
103676649  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
103673102  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
103660837  SOD2; superoxide dismutase [Mn], mitochondrial isoform X1 [KO:K04564] [EC:1.15.1.1]
103672204  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
103664585  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
103681951  GPX5; epididymal secretory glutathione peroxidase isoform X1 [KO:K00432] [EC:1.11.1.9]
103681952  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
103675099  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
103663677  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
103670709  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
103657972  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
103660368  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
103657107  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
103657211  BBC3; bcl-2-binding component 3 [KO:K10132]
804861  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
804862  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
804863  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
804867  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
804868  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
804873  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
804872  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
103679522  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
103662415  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943] [EC:7.1.1.2]
103680708  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
103677743  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
103657024  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
103668224  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
103671508  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
121102487  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
103663752  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
103672884  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
103656470  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
103658820  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
103676131  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
103661190  cytochrome c oxidase subunit NDUFA4-like [KO:K03948]
103676612  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
103674068  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
103681725  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
103670360  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
103675753  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial isoform X1 [KO:K03953]
103662822  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
103668899  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
103681700  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
103660109  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
103668219  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12-like [KO:K11352]
103674915  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
103673805  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
103669030  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
103659737  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
103678783  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
103676116  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X2 [KO:K03961]
103667024  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
103682114  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
103677527  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
103661621  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
103668772  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
103669950  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X2 [KO:K11351]
103659788  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
103672016  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935] [EC:7.1.1.2]
103672304  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
103664628  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
103666354  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
103679235  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
103659515  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial-like isoform X1 [KO:K03939]
103681635  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
103679622  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
103657962  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
103678138  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
103679246  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
103666614  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
103672010  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
103673823  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
804871  CYTB; cytochrome b [KO:K00412]
103656658  cytochrome c1, heme protein, mitochondrial [KO:K00413]
103674845  cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414]
103668880  cytochrome b-c1 complex subunit 2, mitochondrial isoform X1 [KO:K00415]
103663786  cytochrome b-c1 complex subunit 6, mitochondrial [KO:K00416]
103670755  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
103661483  cytochrome b-c1 complex subunit 7 [KO:K00417]
103674244  cytochrome b-c1 complex subunit 7-like [KO:K00417]
103663827  cytochrome b-c1 complex subunit 8 isoform X2 [KO:K00418]
103659249  cytochrome b-c1 complex subunit 9 [KO:K00419]
103681641  cytochrome b-c1 complex subunit 10 [KO:K00420]
804869  COX3; cytochrome c oxidase subunit III [KO:K02262]
804864  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
804870  COX2; cytochrome c oxidase subunit II [KO:K02261]
103673835  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
103674346  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
103679121  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
103671191  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
103679714  cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
103657893  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
103678327  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
103673149  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
103680303  cytochrome c oxidase subunit 6B2 isoform X1 [KO:K02267]
103682512  cytochrome c oxidase subunit 6B1 [KO:K02267]
103675859  cytochrome c oxidase subunit 6B1-like [KO:K02267]
103661502  cytochrome c oxidase subunit 6C [KO:K02268]
103657485  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
103682538  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
103671542  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
103675819  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
103672701  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
103656462  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
103672146  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
103658816  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
103679417  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
103663788  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
103656310  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
103676258  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X2 [KO:K02136]
103681631  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
103670961  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
804865  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
103675040  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
103660617  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
103659901  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
103675541  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
103665721  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
103673122  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
103673070  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
804866  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
103663818  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
103667846  VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040]
103677056  VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041]
103656991  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
103681007  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
103677737  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
103666331  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
103668033  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
103658902  cytochrome c [KO:K08738]
103662923  cytochrome c-like [KO:K08738]
121104237  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
103660136  APAF1; LOW QUALITY PROTEIN: apoptotic protease-activating factor 1 [KO:K02084]
103666628  CASP9; caspase-9 isoform X2 [KO:K04399] [EC:3.4.22.62]
103666073  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
103674585  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
103660313  PSMA4; proteasome subunit alpha type-4 isoform X1 [KO:K02728] [EC:3.4.25.1]
103664901  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
103660584  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
103674995  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
103682383  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
103673363  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
103660341  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
103670332  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
103661230  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
103666399  PSMB2; proteasome subunit beta type-2 isoform X2 [KO:K02734] [EC:3.4.25.1]
103681035  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
103660779  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
103679757  proteasome subunit beta type-1-like [KO:K02732] [EC:3.4.25.1]
103667513  PSMB4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
103658639  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
103670329  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
103661036  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
103672921  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
103672308  PSMC3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
103665634  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
103676082  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
103662680  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032]
103662209  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
103661161  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
103665659  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035]
103667431  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
103674226  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
103668940  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 6-like [KO:K03037]
121106099  LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 6-like [KO:K03037]
103673708  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
103671026  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
103667517  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
103659502  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
103661007  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
103657400  26S proteasome complex subunit SEM1-like [KO:K10881]
103658750  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
103661092  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
103665903  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
103675245  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
103663496  MAP3K5; mitogen-activated protein kinase kinase kinase 5 isoform X2 [KO:K04426] [EC:2.7.11.25]
103681888  MAP2K7; LOW QUALITY PROTEIN: dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
103666660  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
103678902  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
103658551  ULK2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
103675502  ATG101; autophagy-related protein 101 isoform X1 [KO:K19730]
103672325  ATG13; autophagy-related protein 13 [KO:K08331]
103677420  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
103672328  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
103668942  BECN2; beclin-2 [KO:K08334]
103672587  BECN1; beclin-1 isoform X2 [KO:K08334]
103679280  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
103674591  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
103667996  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
103663663  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
103673638  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
103679563  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
103665675  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908]
103670082  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [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
umr00190  Oxidative phosphorylation
umr03020  RNA polymerase
umr03022  Basal transcription factors
umr03050  Proteasome
umr04020  Calcium signaling pathway
umr04115  p53 signaling pathway
umr04140  Autophagy - animal
umr04141  Protein processing in endoplasmic reticulum
umr04144  Endocytosis
umr04210  Apoptosis
umr04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

DBGET integrated database retrieval system