KEGG   PATHWAY: mpuf05016
Entry
mpuf05016                   Pathway                                
Name
Huntington disease - Mustela putorius furo (domestic ferret)
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
mpuf05016  Huntington disease
mpuf05016

Organism
Mustela putorius furo (domestic ferret) [GN:mpuf]
Gene
101691008  CLTA; clathrin light chain A isoform X1 [KO:K04644]
101679889  CLTB; clathrin light chain B isoform X1 [KO:K04645]
101680653  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
101688607  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
101677512  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
101672912  AP2A2; AP-2 complex subunit alpha-2 [KO:K11824]
101685751  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
101670408  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
101674099  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
101671888  AP2S1; AP-2 complex subunit sigma [KO:K11827]
101687209  HTT; huntingtin [KO:K04533]
101672848  DCTN1; dynactin subunit 1 isoform X7 [KO:K04648]
101688644  DCTN2; dynactin subunit 2 isoform X1 [KO:K10424]
101682549  DCTN3; dynactin subunit 3 isoform X2 [KO:K10425]
101677994  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
101680068  DCTN5; dynactin subunit 5 [KO:K10427]
101686473  DCTN6; dynactin subunit 6 [KO:K10428]
101678441  ACTR1A; alpha-centractin [KO:K16575]
101684030  ACTR1B; beta-centractin isoform X1 [KO:K16575]
101686683  ACTR10; actin-related protein 10 [KO:K16576]
101687210  LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
101691572  DNAH7; dynein heavy chain 7, axonemal [KO:K10408]
101679291  DNAH12; dynein heavy chain 12, axonemal isoform X1 [KO:K10408]
101673236  DNAH11; dynein heavy chain 11, axonemal isoform X1 [KO:K10408]
101673976  DNAH1; dynein heavy chain 1, axonemal isoform X1 [KO:K10408]
101674259  DNAH9; dynein heavy chain 9, axonemal isoform X4 [KO:K10408]
101693416  DNAH6; dynein heavy chain 6, axonemal [KO:K10408]
101674625  DNAH5; dynein heavy chain 5, axonemal [KO:K10408]
101680905  DNAH14; dynein heavy chain 14, axonemal isoform X5 [KO:K10408]
101674881  DNAH17; dynein heavy chain 17, axonemal isoform X5 [KO:K10408]
101682048  DNAH8; dynein heavy chain 8, axonemal isoform X1 [KO:K10408]
101682372  DNAH10; dynein heavy chain 10, axonemal isoform X1 [KO:K10408]
101677023  DNAH2; dynein heavy chain 2, axonemal isoform X1 [KO:K10408]
101671134  DNAH3; dynein heavy chain 3, axonemal isoform X1 [KO:K10408]
101692651  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
101679241  DNAI1; dynein intermediate chain 1, axonemal isoform X2 [KO:K10409]
101682859  DNAI2; dynein intermediate chain 2, axonemal [KO:K11143]
101679103  DNAL1; dynein light chain 1, axonemal isoform X2 [KO:K10411]
101672760  DNAL4; dynein light chain 4, axonemal [KO:K10412]
101678877  tubulin alpha-8 chain [KO:K07374]
101692890  tubulin alpha-1A chain-like [KO:K07374]
101679966  tubulin alpha-1C chain [KO:K07374]
101680178  TUBAL3; tubulin alpha chain-like 3 [KO:K07374]
101680276  tubulin alpha-1B chain [KO:K07374]
101675663  TUBA3E; tubulin alpha-3E chain isoform X1 [KO:K07374]
101689201  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
101677236  TUBA4A; tubulin alpha-4A chain [KO:K07374]
101685931  TUBB6; tubulin beta-6 chain [KO:K07375]
101686650  tubulin beta-2B chain [KO:K07375]
101680362  TUBB3; tubulin beta-3 chain [KO:K07375]
101693433  TUBB4A; tubulin beta-4A chain [KO:K07375]
101674529  TUBB; tubulin beta chain [KO:K07375]
101670624  tubulin beta chain [KO:K07375]
101671254  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
101691057  TUBB4B; tubulin beta-4B chain isoform X1 [KO:K07375]
101672072  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
101681349  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
101676423  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
101672332  KIF5B; kinesin-1 heavy chain [KO:K10396]
101689294  KIF5A; kinesin heavy chain isoform 5A [KO:K10396]
101684053  KIF5C; kinesin heavy chain isoform 5C [KO:K10396]
101685097  KLC1; kinesin light chain 1 isoform X7 [KO:K10407]
101693620  KLC3; kinesin light chain 3 [KO:K10407]
101687767  KLC2; kinesin light chain 2 [KO:K10407]
101676637  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
101681973  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
101678102  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
101690858  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
101683208  GRM5; metabotropic glutamate receptor 5 [KO:K04604]
101674115  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634]
101679345  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
101673753  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
101687768  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
101678347  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X2 [KO:K05858] [EC:3.1.4.11]
101682413  STX1A; syntaxin-1A [KO:K04560]
101673094  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X2 [KO:K04849]
101683487  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
101683074  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
101677799  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
101677223  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
101691708  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
101694457  GRIA2; glutamate receptor 2 isoform X5 [KO:K05198]
101674543  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
101684476  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
101692410  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
101680500  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
101687335  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
101677375  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
101691035  REST; RE1-silencing transcription factor isoform X2 [KO:K09222]
101683686  SIN3A; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
101676208  RCOR1; REST corepressor 1 [KO:K11829]
101680954  HDAC2; histone deacetylase 2 isoform X2 [KO:K06067] [EC:3.5.1.98]
101681953  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
101687537  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
101690726  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
101689181  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
101672034  POLR2D; DNA-directed RNA polymerase II subunit RPB4 isoform X2 [KO:K03012]
101678669  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
101684957  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
101685361  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
101684197  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
101681566  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
101693232  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
101687038  BDNF; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
101686721  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
101681230  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
101685341  TAF4B; LOW QUALITY PROTEIN: transcription initiation factor TFIID subunit 4B [KO:K03129]
101676728  TAF4; transcription initiation factor TFIID subunit 4 isoform X1 [KO:K03129]
101672089  TBP; TATA-box-binding protein [KO:K03120]
101684941  TBPL2; TATA box-binding protein-like protein 2 [KO:K03120]
101677424  TBPL1; TATA box-binding protein-like protein 1 [KO:K03120]
101686835  SP1; transcription factor Sp1 [KO:K04684]
101677618  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
101685244  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
101679245  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 isoform X1 [KO:K09048]
101688320  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X2 [KO:K09048]
101681981  CREB3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
101684712  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
101677234  CREB5; cyclic AMP-responsive element-binding protein 5 [KO:K09047]
101689735  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
101688974  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
101672042  TFAM; transcription factor A, mitochondrial [KO:K11830]
101679611  NRF1; nuclear respiratory factor 1 [KO:K11831]
101670031  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
101678830  SOD2; superoxide dismutase [Mn], mitochondrial isoform X1 [KO:K04564] [EC:1.15.1.1]
101671982  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
101680193  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
101686834  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
101688198  GPX5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
101675537  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
101682223  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
101689040  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
101684769  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
101677794  TP53; cellular tumor antigen p53 [KO:K04451]
101686802  BAX; apoptosis regulator BAX isoform X1 [KO:K02159]
101692767  BBC3; bcl-2-binding component 3 [KO:K10132]
101677220  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
101694351  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
101679801  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944]
101683103  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
101681305  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
101671885  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
101678258  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
101678946  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
101671166  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
101688416  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
101673253  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
101686920  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
101675000  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
101680168  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
101693615  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
101679477  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
101682163  acyl carrier protein, mitochondrial [KO:K03955]
101684673  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
101674482  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
101679389  NDUFA13; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
101694062  NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
101674641  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958]
101683892  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
101691663  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
101687434  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
101688606  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
101675882  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
101675804  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
101682240  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
101681324  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
101682983  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
101693700  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
101688652  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
101686313  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
101674933  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
101684469  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
101692362  NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial-like [KO:K03939]
101688718  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
101674706  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940] [EC:7.1.1.2]
101679983  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
101691647  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
101678403  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
101678731  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
101692858  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
101685355  LOW QUALITY PROTEIN: succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236]
101680264  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
101694292  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
101674608  cytochrome c1, heme protein, mitochondrial [KO:K00413]
101694055  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
101672152  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
101685926  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
101681748  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
101680018  cytochrome b-c1 complex subunit 7 [KO:K00417]
101683221  cytochrome b-c1 complex subunit 8 [KO:K00418]
101677535  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
101690736  cytochrome b-c1 complex subunit 10 [KO:K00420]
101680163  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
101676075  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
101679783  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
101684324  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
101679257  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
106003852  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
101689659  cytochrome c oxidase subunit 6B1 [KO:K02267]
101670731  cytochrome c oxidase subunit 6B2 [KO:K02267]
101693169  cytochrome c oxidase subunit 6C [KO:K02268]
101672219  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
101693652  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
101682432  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
101672021  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
101691909  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
101673376  ATP5A1; ATP synthase subunit alpha, mitochondrial [KO:K02132]
101670112  ATP5B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
101689814  ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
101677101  ATP5D; ATP synthase subunit delta, mitochondrial [KO:K02134]
101683533  ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
101672934  ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128]
101673943  ATP5G2; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
106005210  LOW QUALITY PROTEIN: uncharacterized protein LOC106005210 [KO:K02128]
106006495  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
101690076  ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
101693434  ATP5H; ATP synthase subunit d, mitochondrial [KO:K02138]
101677485  ATP5O; ATP synthase subunit O, mitochondrial [KO:K02137]
101678985  ATP5J; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
101679422  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
101674296  VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040]
101687790  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
101694615  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
101688977  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
101689099  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
101683729  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
101676150  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
106003371  cytochrome c, testis-specific [KO:K08738]
101690488  cytochrome c [KO:K08738]
101691091  APAF1; apoptotic protease-activating factor 1 [KO:K02084]
101681290  CASP9; caspase-9 [KO:K04399] [EC:3.4.22.62]
101694465  PSMA6; proteasome subunit alpha type-6 isoform X1 [KO:K02730] [EC:3.4.25.1]
101682226  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
101688003  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
101685934  PSMA8; proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
101678084  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
101688820  PSMA5; proteasome subunit alpha type-5 [KO:K02729] [EC:3.4.25.1]
101691417  LOW QUALITY PROTEIN: proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
101693287  PSMA1; proteasome subunit alpha type-1 [KO:K02725] [EC:3.4.25.1]
101681087  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
101689827  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
101673192  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
101682336  PSMB3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
101681952  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
101684806  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
101677063  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
101692243  PSMB4; proteasome subunit beta type-4 isoform X3 [KO:K02736] [EC:3.4.25.1]
101689387  26S protease regulatory subunit 7-like [KO:K03061]
101691078  PSMC2; 26S protease regulatory subunit 7 [KO:K03061]
101691713  PSMC1; 26S protease regulatory subunit 4 [KO:K03062]
101689211  PSMC4; 26S protease regulatory subunit 6B [KO:K03063]
101689832  PSMC6; 26S protease regulatory subunit 10B isoform X1 [KO:K03064]
101688313  PSMC3; 26S protease regulatory subunit 6A [KO:K03065]
106004683  ATP-dependent zinc metalloprotease YME1L1-like [KO:K03066]
101671803  PSMC5; 26S protease regulatory subunit 8 isoform X1 [KO:K03066]
101679423  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
101677647  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
101689542  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
101681801  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
101682559  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
101682352  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
101670551  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
101690051  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
101674526  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X2 [KO:K03039]
101672325  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
101670572  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
101687128  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
101687725  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
101683996  SHFM1; 26S proteasome complex subunit DSS1 [KO:K10881]
101688180  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
101689378  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
101691230  TRAF2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
101686402  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
101690814  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
101676245  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
101670633  ULK1; serine/threonine-protein kinase ULK1 [KO:K21357] [EC:2.7.11.1]
101682377  ULK2; LOW QUALITY PROTEIN: serine/threonine-protein kinase ULK2 [KO:K08269] [EC:2.7.11.1]
101682698  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
101675999  ATG101; autophagy-related protein 101 [KO:K19730]
101679604  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
101692151  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
101680997  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
101680757  BECN1; beclin-1 isoform X2 [KO:K08334]
101684048  BECN2; beclin-2 [KO:K08334]
101684646  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
101681569  NRBF2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
101679553  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
101691773  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
101674575  ATG2A; autophagy-related protein 2 homolog A isoform X2 [KO:K17906]
101688271  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
101676578  WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [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
mpuf00190  Oxidative phosphorylation
mpuf03020  RNA polymerase
mpuf03022  Basal transcription factors
mpuf03050  Proteasome
mpuf04020  Calcium signaling pathway
mpuf04115  p53 signaling pathway
mpuf04140  Autophagy - animal
mpuf04141  Protein processing in endoplasmic reticulum
mpuf04144  Endocytosis
mpuf04210  Apoptosis
mpuf04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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