KEGG   PATHWAY: hsa05016Help
Entry
hsa05016                    Pathway                                

Name
Huntington's disease - Homo sapiens (human)
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 IT15gene, which results in a long stretch of polyglutamine close to the amino-terminus of the HD protein huntingtin (Htt). Mutant Htt (mHtt) has effects both in the cytoplasm and in the nucleus. In the cytoplasm, full-length mHtt can interfere with BDNF vesicular transport on microtubules. This mutant protein also may lead to abnormal endocytosis and secretion in neurons, because normal Htt form a complex with the proteins Hip1, clathrin and AP2 that are involved in endocytosis. In addition, mHtt affects Ca2+ signaling by sensitizing InsP3R1 to activation by InsP3, stimulating NMDAR activity, and destabilizing mitochondrial Ca2+ handling. The mHtt translocates to the nucleus, where it forms intranuclear inclusions. Nuclear toxicity is believed to be caused by interference with gene transcription, leading to loss of transcription of neuroprotective molecules such as BDNF. While mHtt binds to p53 and upregulates levels of nuclear p53 as well as p53 transcriptional activity. Augmented p53 mediates mitochondrial dysfunction.
Class
Human Diseases; Neurodegenerative diseases
BRITE hierarchy
Pathway map
Huntington's disease
hsa05016

All organismsOrtholog table
Module
RNA polymerase II, eukaryotes [PATH:hsa05016]
Disease
H00059  
Huntington's disease (HD)
Organism
Homo sapiens (human) [GN:hsa]
Gene
1211  
K04644 clathrin light chain A | (RefSeq) CLTA; clathrin light chain A [KO:K04644]
1212  
K04645 clathrin light chain B | (RefSeq) CLTB; clathrin light chain B [KO:K04645]
1213  
K04646 clathrin heavy chain | (RefSeq) CLTC; clathrin heavy chain [KO:K04646]
8218  
K04646 clathrin heavy chain | (RefSeq) CLTCL1; clathrin heavy chain like 1 [KO:K04646]
3092  
K04559 huntingtin interacting protein 1 | (RefSeq) HIP1; huntingtin interacting protein 1 [KO:K04559]
161  
K11824 AP-2 complex subunit alpha | (RefSeq) AP2A2; adaptor related protein complex 2 alpha 2 subunit [KO:K11824]
160  
K11824 AP-2 complex subunit alpha | (RefSeq) AP2A1; adaptor related protein complex 2 alpha 1 subunit [KO:K11824]
163  
K11825 AP-2 complex subunit beta-1 | (RefSeq) AP2B1; adaptor related protein complex 2 beta 1 subunit [KO:K11825]
1173  
K11826 AP-2 complex subunit mu-1 | (RefSeq) AP2M1; adaptor related protein complex 2 mu 1 subunit [KO:K11826]
1175  
K11827 AP-2 complex subunit sigma-1 | (RefSeq) AP2S1; adaptor related protein complex 2 sigma 1 subunit [KO:K11827]
3064  
K04533 huntingtin | (RefSeq) HTT; huntingtin [KO:K04533]
55081  
K04638 intraflagellar transport protein 57 | (RefSeq) IFT57; intraflagellar transport 57 [KO:K04638]
841  
K04398 caspase 8 [EC:3.4.22.61] | (RefSeq) CASP8; caspase 8 [KO:K04398] [EC:3.4.22.61]
836  
K02187 caspase 3 [EC:3.4.22.56] | (RefSeq) CASP3; caspase 3 [KO:K02187] [EC:3.4.22.56]
2915  
K04604 metabotropic glutamate receptor 5 | (RefSeq) GRM5; glutamate metabotropic receptor 5 [KO:K04604]
2776  
K04634 guanine nucleotide-binding protein G(q) subunit alpha | (RefSeq) GNAQ; G protein subunit alpha q [KO:K04634]
23236  
K05858 phosphatidylinositol phospholipase C; phospholipase C beta 1 [KO:K05858] [EC:3.1.4.11]
5330  
K05858 phosphatidylinositol phospholipase C; phospholipase C beta 2 [KO:K05858] [EC:3.1.4.11]
5331  
K05858 phosphatidylinositol phospholipase C; phospholipase C beta 3 [KO:K05858] [EC:3.1.4.11]
5332  
K05858 phosphatidylinositol phospholipase C; phospholipase C beta 4 [KO:K05858] [EC:3.1.4.11]
2902  
K05208 glutamate receptor ionotropic; glutamate ionotropic receptor NMDA type subunit 1 [KO:K05208]
2904  
K05210 glutamate receptor ionotropic; glutamate ionotropic receptor NMDA type subunit 2B [KO:K05210]
3708  
K04958 inositol 1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
1742  
K11828 discs large protein 4 | (RefSeq) DLG4; discs large MAGUK scaffold protein 4 [KO:K11828]
7052  
K05625 transglutaminase 2 [EC:2.3.2.13] | (RefSeq) TGM2; transglutaminase 2 [KO:K05625] [EC:2.3.2.13]
5978  
K09222 RE1-silencing transcription factor | (RefSeq) REST; RE1 silencing transcription factor [KO:K09222]
25942  
K11644 paired amphipathic helix protein Sin3a | (RefSeq) SIN3A; SIN3 transcription regulator family member A [KO:K11644]
23186  
K11829 REST corepressor 1 | (RefSeq) RCOR1; REST corepressor 1 [KO:K11829]
3065  
K06067 histone deacetylase 1/2 [EC:3.5.1.98] | (RefSeq) HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
3066  
K06067 histone deacetylase 1/2 [EC:3.5.1.98] | (RefSeq) HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
5430  
K03006 DNA-directed RNA polymerase II subunit RPB1 [EC:2.7.7.6] | (RefSeq) POLR2A; RNA polymerase II subunit A [KO:K03006] [EC:2.7.7.6]
5431  
K03010 DNA-directed RNA polymerase II subunit RPB2 [EC:2.7.7.6] | (RefSeq) POLR2B; RNA polymerase II subunit B [KO:K03010] [EC:2.7.7.6]
5432  
K03011 DNA-directed RNA polymerase II subunit RPB3 | (RefSeq) POLR2C; RNA polymerase II subunit C [KO:K03011]
5433  
K03012 DNA-directed RNA polymerase II subunit RPB4 | (RefSeq) POLR2D; RNA polymerase II subunit D [KO:K03012]
5434  
K03013 DNA-directed RNA polymerases I; RNA polymerase II subunit E [KO:K03013]
5435  
K03014 DNA-directed RNA polymerases I; RNA polymerase II subunit F [KO:K03014]
5436  
K03015 DNA-directed RNA polymerase II subunit RPB7 | (RefSeq) POLR2G; RNA polymerase II subunit G [KO:K03015]
5437  
K03016 DNA-directed RNA polymerases I; RNA polymerase II subunit H [KO:K03016]
5438  
K03017 DNA-directed RNA polymerase II subunit RPB9 | (RefSeq) POLR2I; RNA polymerase II subunit I [KO:K03017]
5441  
K03007 DNA-directed RNA polymerases I; RNA polymerase II subunit L [KO:K03007]
5439  
K03008 DNA-directed RNA polymerase II subunit RPB11 | (RefSeq) POLR2J; RNA polymerase II subunit J [KO:K03008]
548644  
K03008 DNA-directed RNA polymerase II subunit RPB11 | (RefSeq) POLR2J3; RNA polymerase II subunit J3 [KO:K03008]
246721  
K03008 DNA-directed RNA polymerase II subunit RPB11 | (RefSeq) POLR2J2; RNA polymerase II subunit J2 [KO:K03008]
5440  
K03009 DNA-directed RNA polymerases I; RNA polymerase II subunit K [KO:K03009]
627  
K04355 brain-derived neurotrophic factor | (RefSeq) BDNF; brain derived neurotrophic factor [KO:K04355]
1639  
K04648 dynactin 1 | (RefSeq) DCTN1; dynactin subunit 1 [KO:K04648]
10540  
K10424 dynactin 2 | (RefSeq) DCTN2; dynactin subunit 2 [KO:K10424]
51164  
K10426 dynactin 4 | (RefSeq) DCTN4; dynactin subunit 4 [KO:K10426]
9001  
K04647 huntingtin-associated protein 1 | (RefSeq) HAP1; huntingtin associated protein 1 [KO:K04647]
25981  
K10408 dynein heavy chain; dynein axonemal heavy chain 1 [KO:K10408]
55567  
K10408 dynein heavy chain; dynein axonemal heavy chain 3 [KO:K10408]
146754  
K10408 dynein heavy chain; dynein axonemal heavy chain 2 [KO:K10408]
56171  
K10408 dynein heavy chain; dynein axonemal heavy chain 7 [KO:K10408]
1767  
K10408 dynein heavy chain; dynein axonemal heavy chain 5 [KO:K10408]
1768  
K10408 dynein heavy chain; dynein axonemal heavy chain 6 [KO:K10408]
1770  
K10408 dynein heavy chain; dynein axonemal heavy chain 9 [KO:K10408]
8701  
K10408 dynein heavy chain; dynein axonemal heavy chain 11 [KO:K10408]
201625  
K10408 dynein heavy chain; dynein axonemal heavy chain 12 [KO:K10408]
127602  
K10408 dynein heavy chain; dynein axonemal heavy chain 14 [KO:K10408]
1769  
K10408 dynein heavy chain; dynein axonemal heavy chain 8 [KO:K10408]
196385  
K10408 dynein heavy chain; dynein axonemal heavy chain 10 [KO:K10408]
8632  
K10408 dynein heavy chain; dynein axonemal heavy chain 17 [KO:K10408]
7802  
K10410 dynein light intermediate chain; dynein axonemal light intermediate chain 1 [KO:K10410]
27019  
K10409 dynein intermediate chain 1; dynein axonemal intermediate chain 1 [KO:K10409]
64446  
K11143 dynein intermediate chain 2; dynein axonemal intermediate chain 2 [KO:K11143]
83544  
K10411 dynein light chain 1; dynein axonemal light chain 1 [KO:K10411]
10126  
K10412 dynein light chain 4; dynein axonemal light chain 4 [KO:K10412]
1387  
K04498 E1A/CREB-binding protein [EC:2.3.1.48] | (RefSeq) CREBBP; CREB binding protein [KO:K04498] [EC:2.3.1.48]
2033  
K04498 E1A/CREB-binding protein [EC:2.3.1.48] | (RefSeq) EP300; E1A binding protein p300 [KO:K04498] [EC:2.3.1.48]
6875  
K03129 transcription initiation factor TFIID subunit 4 | (RefSeq) TAF4B; TATA-box binding protein associated factor 4b [KO:K03129]
6874  
K03129 transcription initiation factor TFIID subunit 4 | (RefSeq) TAF4; TATA-box binding protein associated factor 4 [KO:K03129]
387332  
K03120 transcription initiation factor TFIID TATA-box-binding protein | (RefSeq) TBPL2; TATA-box binding protein like 2 [KO:K03120]
9519  
K03120 transcription initiation factor TFIID TATA-box-binding protein | (RefSeq) TBPL1; TATA-box binding protein like 1 [KO:K03120]
6908  
K03120 transcription initiation factor TFIID TATA-box-binding protein | (RefSeq) TBP; TATA-box binding protein [KO:K03120]
6667  
K04684 transcription factor Sp1 | (RefSeq) SP1; Sp1 transcription factor [KO:K04684]
1385  
K05870 cyclic AMP-responsive element-binding protein 1 | (RefSeq) CREB1; cAMP responsive element binding protein 1 [KO:K05870]
10488  
K09048 cyclic AMP-responsive element-binding protein 3 | (RefSeq) CREB3; cAMP responsive element binding protein 3 [KO:K09048]
90993  
K09048 cyclic AMP-responsive element-binding protein 3 | (RefSeq) CREB3L1; cAMP responsive element binding protein 3 like 1 [KO:K09048]
64764  
K09048 cyclic AMP-responsive element-binding protein 3 | (RefSeq) CREB3L2; cAMP responsive element binding protein 3 like 2 [KO:K09048]
84699  
K09048 cyclic AMP-responsive element-binding protein 3 | (RefSeq) CREB3L3; cAMP responsive element binding protein 3 like 3 [KO:K09048]
148327  
K09048 cyclic AMP-responsive element-binding protein 3 | (RefSeq) CREB3L4; cAMP responsive element binding protein 3 like 4 [KO:K09048]
9586  
K09047 cyclic AMP-responsive element-binding protein 5 | (RefSeq) CREB5; cAMP responsive element binding protein 5 [KO:K09047]
10891  
K07202 alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha | (RefSeq) PPARGC1A; PPARG coactivator 1 alpha [KO:K07202]
5468  
K08530 peroxisome proliferator-activated receptor gamma | (RefSeq) PPARG; peroxisome proliferator activated receptor gamma [KO:K08530]
7019  
K11830 transcription factor A; transcription factor A, mitochondrial [KO:K11830]
4899  
K11831 nuclear respiratory factor 1 | (RefSeq) NRF1; nuclear respiratory factor 1 [KO:K11831]
6647  
K04565 superoxide dismutase; superoxide dismutase 1, soluble [KO:K04565] [EC:1.15.1.1]
6648  
K04564 superoxide dismutase; superoxide dismutase 2, mitochondrial [KO:K04564] [EC:1.15.1.1]
7350  
K08769 solute carrier family 25 (mitochondrial uncoupling protein); uncoupling protein 1 [KO:K08769]
7157  
K04451 tumor protein p53 | (RefSeq) TP53; tumor protein p53 [KO:K04451]
581  
K02159 apoptosis regulator BAX | (RefSeq) BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
27113  
K10132 Bcl-2-binding component 3 | (RefSeq) BBC3; BCL2 binding component 3 [KO:K10132]
4723  
K03942 NADH dehydrogenase (ubiquinone) flavoprotein 1 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFV1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:1.6.99.3 1.6.5.3]
4729  
K03943 NADH dehydrogenase (ubiquinone) flavoprotein 2 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFV2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:1.6.99.3 1.6.5.3]
4731  
K03944 NADH dehydrogenase (ubiquinone) flavoprotein 3 | (RefSeq) NDUFV3; NADH:ubiquinone oxidoreductase subunit V3 [KO:K03944]
4694  
K03945 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 1 | (RefSeq) NDUFA1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
4695  
K03946 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 2 | (RefSeq) NDUFA2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
4696  
K03947 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 3 | (RefSeq) NDUFA3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
4697  
K03948 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4 | (RefSeq) NDUFA4; NDUFA4, mitochondrial complex associated [KO:K03948]
56901  
K03948 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4 | (RefSeq) NDUFA4L2; NDUFA4, mitochondrial complex associated like 2 [KO:K03948]
4698  
K03949 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 5 | (RefSeq) NDUFA5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
4700  
K03950 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 6 | (RefSeq) NDUFA6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
4701  
K03951 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 7 | (RefSeq) NDUFA7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
4702  
K03952 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 8 | (RefSeq) NDUFA8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
4704  
K03953 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 9 | (RefSeq) NDUFA9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
4705  
K03954 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 10 | (RefSeq) NDUFA10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
4706  
K03955 NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1 | (RefSeq) NDUFAB1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
126328  
K03956 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 11 | (RefSeq) NDUFA11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
55967  
K11352 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 12 | (RefSeq) NDUFA12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
51079  
K11353 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13 | (RefSeq) NDUFA13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
4707  
K03957 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 1 | (RefSeq) NDUFB1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
4708  
K03958 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 2 | (RefSeq) NDUFB2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
4709  
K03959 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 3 | (RefSeq) NDUFB3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
4710  
K03960 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 4 | (RefSeq) NDUFB4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
4711  
K03961 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 5 | (RefSeq) NDUFB5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
4712  
K03962 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6 | (RefSeq) NDUFB6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
4713  
K03963 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7 | (RefSeq) NDUFB7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
4714  
K03964 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8 | (RefSeq) NDUFB8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
4715  
K03965 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9 | (RefSeq) NDUFB9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
4716  
K03966 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10 | (RefSeq) NDUFB10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
54539  
K11351 NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11 | (RefSeq) NDUFB11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
4719  
K03934 NADH dehydrogenase (ubiquinone) Fe-S protein 1 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFS1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:1.6.99.3 1.6.5.3]
4720  
K03935 NADH dehydrogenase (ubiquinone) Fe-S protein 2 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFS2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:1.6.99.3 1.6.5.3]
4722  
K03936 NADH dehydrogenase (ubiquinone) Fe-S protein 3 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFS3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:1.6.99.3 1.6.5.3]
4724  
K03937 NADH dehydrogenase (ubiquinone) Fe-S protein 4 | (RefSeq) NDUFS4; NADH:ubiquinone oxidoreductase subunit S4 [KO:K03937]
4725  
K03938 NADH dehydrogenase (ubiquinone) Fe-S protein 5 | (RefSeq) NDUFS5; NADH:ubiquinone oxidoreductase subunit S5 [KO:K03938]
4726  
K03939 NADH dehydrogenase (ubiquinone) Fe-S protein 6 | (RefSeq) NDUFS6; NADH:ubiquinone oxidoreductase subunit S6 [KO:K03939]
374291  
K03940 NADH dehydrogenase (ubiquinone) Fe-S protein 7 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFS7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:1.6.99.3 1.6.5.3]
4728  
K03941 NADH dehydrogenase (ubiquinone) Fe-S protein 8 [EC:1.6.5.3 1.6.99.3] | (RefSeq) NDUFS8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:1.6.99.3 1.6.5.3]
4717  
K03967 NADH dehydrogenase (ubiquinone) 1 subunit C1 | (RefSeq) NDUFC1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
4718  
K03968 NADH dehydrogenase (ubiquinone) 1 subunit C2 | (RefSeq) NDUFC2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
K03968 NADH dehydrogenase (ubiquinone) 1 subunit C2 | (RefSeq) NDUFC2-KCTD14; NDUFC2-KCTD14 readthrough [KO:K03968]
6389  
K00234 succinate dehydrogenase (ubiquinone) flavoprotein subunit [EC:1.3.5.1] | (RefSeq) SDHA; succinate dehydrogenase complex flavoprotein subunit A [KO:K00234] [EC:1.3.5.1]
6390  
K00235 succinate dehydrogenase (ubiquinone) iron-sulfur subunit [EC:1.3.5.1] | (RefSeq) SDHB; succinate dehydrogenase complex iron sulfur subunit B [KO:K00235] [EC:1.3.5.1]
6391  
K00236 succinate dehydrogenase (ubiquinone) cytochrome b560 subunit | (RefSeq) SDHC; succinate dehydrogenase complex subunit C [KO:K00236]
6392  
K00237 succinate dehydrogenase (ubiquinone) membrane anchor subunit | (RefSeq) SDHD; succinate dehydrogenase complex subunit D [KO:K00237]
7386  
K00411 ubiquinol-cytochrome c reductase iron-sulfur subunit [EC:1.10.2.2] | (RefSeq) UQCRFS1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:1.10.2.2]
4519  
K00412 ubiquinol-cytochrome c reductase cytochrome b subunit | (RefSeq) CYTB; cytochrome b [KO:K00412]
1537  
K00413 ubiquinol-cytochrome c reductase cytochrome c1 subunit | (RefSeq) CYC1; cytochrome c1 [KO:K00413]
7384  
K00414 ubiquinol-cytochrome c reductase core subunit 1 | (RefSeq) UQCRC1; ubiquinol-cytochrome c reductase core protein I [KO:K00414]
7385  
K00415 ubiquinol-cytochrome c reductase core subunit 2 | (RefSeq) UQCRC2; ubiquinol-cytochrome c reductase core protein II [KO:K00415]
7388  
K00416 ubiquinol-cytochrome c reductase subunit 6 | (RefSeq) UQCRH; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
440567  
K00416 ubiquinol-cytochrome c reductase subunit 6 | (RefSeq) UQCRHL; ubiquinol-cytochrome c reductase hinge protein like [KO:K00416]
7381  
K00417 ubiquinol-cytochrome c reductase subunit 7 | (RefSeq) UQCRB; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
27089  
K00418 ubiquinol-cytochrome c reductase subunit 8 | (RefSeq) UQCRQ; ubiquinol-cytochrome c reductase complex III subunit VII [KO:K00418]
29796  
K00419 ubiquinol-cytochrome c reductase subunit 9 | (RefSeq) UQCR10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
10975  
K00420 ubiquinol-cytochrome c reductase subunit 10 | (RefSeq) UQCR11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
4514  
K02262 cytochrome c oxidase subunit 3 | (RefSeq) COX3; cytochrome c oxidase III [KO:K02262]
4512  
K02256 cytochrome c oxidase subunit 1 [EC:1.9.3.1] | (RefSeq) COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:1.9.3.1]
4513  
K02261 cytochrome c oxidase subunit 2 | (RefSeq) COX2; cytochrome c oxidase subunit II [KO:K02261]
84701  
K02263 cytochrome c oxidase subunit 4 | (RefSeq) COX4I2; cytochrome c oxidase subunit 4I2 [KO:K02263]
1327  
K02263 cytochrome c oxidase subunit 4 | (RefSeq) COX4I1; cytochrome c oxidase subunit 4I1 [KO:K02263]
9377  
K02264 cytochrome c oxidase subunit 5a | (RefSeq) COX5A; cytochrome c oxidase subunit 5A [KO:K02264]
1329  
K02265 cytochrome c oxidase subunit 5b | (RefSeq) COX5B; cytochrome c oxidase subunit 5B [KO:K02265]
1337  
K02266 cytochrome c oxidase subunit 6a | (RefSeq) COX6A1; cytochrome c oxidase subunit 6A1 [KO:K02266]
1339  
K02266 cytochrome c oxidase subunit 6a | (RefSeq) COX6A2; cytochrome c oxidase subunit 6A2 [KO:K02266]
1340  
K02267 cytochrome c oxidase subunit 6b | (RefSeq) COX6B1; cytochrome c oxidase subunit 6B1 [KO:K02267]
125965  
K02267 cytochrome c oxidase subunit 6b | (RefSeq) COX6B2; cytochrome c oxidase subunit 6B2 [KO:K02267]
1345  
K02268 cytochrome c oxidase subunit 6c | (RefSeq) COX6C; cytochrome c oxidase subunit 6C [KO:K02268]
1346  
K02270 cytochrome c oxidase subunit 7a | (RefSeq) COX7A1; cytochrome c oxidase subunit 7A1 [KO:K02270]
1347  
K02270 cytochrome c oxidase subunit 7a | (RefSeq) COX7A2; cytochrome c oxidase subunit 7A2 [KO:K02270]
9167  
K02270 cytochrome c oxidase subunit 7a | (RefSeq) COX7A2L; cytochrome c oxidase subunit 7A2 like [KO:K02270]
1349  
K02271 cytochrome c oxidase subunit 7b | (RefSeq) COX7B; cytochrome c oxidase subunit 7B [KO:K02271]
170712  
K02271 cytochrome c oxidase subunit 7b | (RefSeq) COX7B2; cytochrome c oxidase subunit 7B2 [KO:K02271]
1350  
K02272 cytochrome c oxidase subunit 7c | (RefSeq) COX7C; cytochrome c oxidase subunit 7C [KO:K02272]
341947  
K02273 cytochrome c oxidase subunit 8 | (RefSeq) COX8C; cytochrome c oxidase subunit 8C [KO:K02273]
1351  
K02273 cytochrome c oxidase subunit 8 | (RefSeq) COX8A; cytochrome c oxidase subunit 8A [KO:K02273]
498  
K02132 F-type H+-transporting ATPase subunit alpha | (RefSeq) ATP5A1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132]
506  
K02133 F-type H+-transporting ATPase subunit beta [EC:3.6.3.14] | (RefSeq) ATP5B; ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide [KO:K02133] [EC:3.6.3.14]
509  
K02136 F-type H+-transporting ATPase subunit gamma | (RefSeq) ATP5C1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136]
513  
K02134 F-type H+-transporting ATPase subunit delta | (RefSeq) ATP5D; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134]
514  
K02135 F-type H+-transporting ATPase subunit epsilon | (RefSeq) ATP5E; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135]
4508  
K02126 F-type H+-transporting ATPase subunit a | (RefSeq) ATP6; ATP synthase F0 subunit 6 [KO:K02126]
515  
K02127 F-type H+-transporting ATPase subunit b | (RefSeq) ATP5F1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit B1 [KO:K02127]
516  
K02128 F-type H+-transporting ATPase subunit c | (RefSeq) ATP5G1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C1 (subunit 9) [KO:K02128]
517  
K02128 F-type H+-transporting ATPase subunit c | (RefSeq) ATP5G2; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C2 (subunit 9) [KO:K02128]
518  
K02128 F-type H+-transporting ATPase subunit c | (RefSeq) ATP5G3; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C3 (subunit 9) [KO:K02128]
10476  
K02138 F-type H+-transporting ATPase subunit d | (RefSeq) ATP5H; ATP synthase, H+ transporting, mitochondrial Fo complex subunit D [KO:K02138]
539  
K02137 F-type H+-transporting ATPase subunit O | (RefSeq) ATP5O; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137]
522  
K02131 F-type H+-transporting ATPase subunit 6 | (RefSeq) ATP5J; ATP synthase, H+ transporting, mitochondrial Fo complex subunit F6 [KO:K02131]
4509  
K02125 F-type H+-transporting ATPase subunit 8 | (RefSeq) ATP8; ATP synthase F0 subunit 8 [KO:K02125]
7416  
K05862 voltage-dependent anion channel protein 1 | (RefSeq) VDAC1; voltage dependent anion channel 1 [KO:K05862]
7417  
K15040 voltage-dependent anion channel protein 2 | (RefSeq) VDAC2; voltage dependent anion channel 2 [KO:K15040]
7419  
K15041 voltage-dependent anion channel protein 3 | (RefSeq) VDAC3; voltage dependent anion channel 3 [KO:K15041]
291  
K05863 solute carrier family 25 (mitochondrial adenine nucleotide translocator); solute carrier family 25 member 4 [KO:K05863]
292  
K05863 solute carrier family 25 (mitochondrial adenine nucleotide translocator); solute carrier family 25 member 5 [KO:K05863]
293  
K05863 solute carrier family 25 (mitochondrial adenine nucleotide translocator); solute carrier family 25 member 6 [KO:K05863]
83447  
K05863 solute carrier family 25 (mitochondrial adenine nucleotide translocator); solute carrier family 25 member 31 [KO:K05863]
10105  
K09565 peptidyl-prolyl isomerase F (cyclophilin D) [EC:5.2.1.8] | (RefSeq) PPIF; peptidylprolyl isomerase F [KO:K09565] [EC:5.2.1.8]
54205  
K08738 cytochrome c | (RefSeq) CYCS; cytochrome c, somatic [KO:K08738]
317  
K02084 apoptotic protease-activating factor | (RefSeq) APAF1; apoptotic peptidase activating factor 1 [KO:K02084]
842  
K04399 caspase 9 [EC:3.4.22.62] | (RefSeq) CASP9; caspase 9 [KO:K04399] [EC:3.4.22.62]
2876  
K00432 glutathione peroxidase [EC:1.11.1.9] | (RefSeq) GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
Compound
C00025  
L-Glutamate
C00076  
Calcium cation
C01245  
D-myo-Inositol 1,4,5-trisphosphate
Reference
  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)
Reference
  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)
Reference
  Authors
Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
Reference
  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)
Reference
  Authors
Bezprozvanny I, Hayden MR
  Title
Deranged neuronal calcium signaling and Huntington disease.
  Journal
Biochem Biophys Res Commun 322:1310-7 (2004)
Reference
  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)
Reference
  Authors
Ross CA.
  Title
Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
  Journal
Neuron 35:819-22 (2002)
Reference
  Authors
Ross CA
  Title
Huntington's disease: new paths to pathogenesis.
  Journal
Cell 118:4-7 (2004)
Reference
  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)
Reference
  Authors
Mattson MP.
  Title
Accomplices to neuronal death.
  Journal
Nature 415:377-9 (2002)
Reference
  Authors
McGill JK, Beal MF
  Title
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Journal
Cell 127:465-8 (2006)
Reference
  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)
Reference
  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)
Reference
  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)
Reference
  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)
KO pathway
 

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