KEGG   PATHWAY: mlk05016
Entry
mlk05016                    Pathway                                
Name
Huntington disease - Mustela lutreola (European mink)
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
mlk05016  Huntington disease
mlk05016

Organism
Mustela lutreola (European mink) [GN:mlk]
Gene
131812901  CLTA; clathrin light chain A isoform X1 [KO:K04644]
131831946  CLTB; clathrin light chain B isoform X1 [KO:K04645]
131811372  CLTCL1; clathrin heavy chain 2 isoform X1 [KO:K04646]
131815835  CLTC; clathrin heavy chain 1 isoform X1 [KO:K04646]
131819667  HIP1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
131818810  AP2A1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
131817922  AP2A2; AP-2 complex subunit alpha-2 isoform X1 [KO:K11824]
131815547  AP2B1; AP-2 complex subunit beta isoform X1 [KO:K11825]
131824799  AP2M1; AP-2 complex subunit mu isoform X1 [KO:K11826]
131818380  AP2S1; AP-2 complex subunit sigma [KO:K11827]
131826799  HTT; huntingtin isoform X1 [KO:K04533]
131840318  DCTN1; dynactin subunit 1 isoform X1 [KO:K04648]
131838556  DCTN2; dynactin subunit 2 [KO:K10424]
131812342  DCTN3; dynactin subunit 3 isoform X1 [KO:K10425]
131831779  DCTN4; dynactin subunit 4 isoform X1 [KO:K10426]
131819984  DCTN5; dynactin subunit 5 [KO:K10427]
131820430  DCTN6; dynactin subunit 6 [KO:K10428]
131828499  ACTR1A; alpha-centractin [KO:K16575]
131808563  ACTR1B; beta-centractin isoform X1 [KO:K16575]
131836617  ACTR10; actin-related protein 10 [KO:K16576]
131816712  HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647]
131840375  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
131811302  DNAH10; dynein axonemal heavy chain 10 isoform X1 [KO:K10408]
131833920  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
131819419  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
131827638  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
131814581  DNAH14; dynein axonemal heavy chain 14 [KO:K10408]
131829488  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
131815514  DNAH2; LOW QUALITY PROTEIN: dynein axonemal heavy chain 2 [KO:K10408]
131824006  DNAH1; dynein axonemal heavy chain 1 isoform X1 [KO:K10408]
131824058  DNAH12; dynein axonemal heavy chain 12 isoform X1 [KO:K10408]
131831589  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
131816853  DNAH17; dynein axonemal heavy chain 17 isoform X1 [KO:K10408]
131816963  DNAH9; dynein axonemal heavy chain 9 isoform X1 [KO:K10408]
131809581  DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
131812422  DNAI1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
131816398  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
131836894  DNAL1; dynein axonemal light chain 1 isoform X1 [KO:K10411]
131839491  DNAL4; dynein axonemal light chain 4 [KO:K10412]
131838055  TUBAL3; LOW QUALITY PROTEIN: tubulin alpha chain-like 3 [KO:K07374]
131838335  TUBA8; tubulin alpha-8 chain isoform X1 [KO:K07374]
131838804  tubulin alpha-1B chain isoform X1 [KO:K07374]
131838805  tubulin alpha-1A chain [KO:K07374]
131838806  tubulin alpha-1C chain isoform X1 [KO:K07374]
131811241  tubulin alpha-3 chain-like [KO:K07374]
131819705  tubulin alpha-1B chain-like [KO:K07374]
131827429  tubulin alpha-4A chain [KO:K07374]
131814751  tubulin alpha-8 chain isoform X1 [KO:K07374]
131824412  tubulin alpha-1A chain-like [KO:K07374]
131810812  tubulin alpha-3 chain [KO:K07374]
131811842  TUBB6; tubulin beta-6 chain isoform X1 [KO:K07375]
131812304  TUBB4B; tubulin beta-4B chain [KO:K07375]
131835737  tubulin beta chain [KO:K07375]
131834412  TUBB; tubulin beta chain [KO:K07375]
131834463  TUBB2A; tubulin beta-2A chain [KO:K07375]
131834464  tubulin beta-2B chain [KO:K07375]
131807671  TUBB1; tubulin beta-1 chain isoform X1 [KO:K07375]
131823379  TUBB4A; tubulin beta-4A chain [KO:K07375]
131818429  TUBB3; tubulin beta-3 chain [KO:K07375]
131828723  MAPK8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
131831160  MAPK10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
131832665  MAPK9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
131838301  KIF5B; kinesin-1 heavy chain [KO:K10396]
131838554  KIF5A; kinesin heavy chain [KO:K10396]
131826995  KIF5C; kinesin heavy chain [KO:K10396]
131837031  KLC1; kinesin light chain 1 isoform X1 [KO:K10407]
131818751  KLC3; kinesin light chain 3 isoform X1 [KO:K10407]
131833990  KLC4; kinesin light chain 4 isoform X1 [KO:K10407]
131815878  KLC2; kinesin light chain 2 isoform X1 [KO:K10407]
131825166  IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638]
131827572  CASP8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
131820467  CASP3; caspase-3 [KO:K02187] [EC:3.4.22.56]
131839461  GRM5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
131812467  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
131836105  PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
131814631  PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 [KO:K05858] [EC:3.1.4.11]
131808069  PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858] [EC:3.1.4.11]
131808080  PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858] [EC:3.1.4.11]
131819520  STX1A; syntaxin-1A [KO:K04560]
131812571  CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849]
131812507  GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
131839014  GRIN2B; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
131824160  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958]
131815750  DLG4; disks large homolog 4 isoform X1 [KO:K11828]
131831806  GRIA1; glutamate receptor 1 isoform X1 [KO:K05197]
131831720  GRIA2; glutamate receptor 2 isoform X1 [KO:K05198]
131821141  GRIA3; glutamate receptor 3 isoform X1 [KO:K05199]
131834835  GRIA4; glutamate receptor 4 isoform X1 [KO:K05200]
131807669  TGM2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
131831664  SLC1A3; excitatory amino acid transporter 1 [KO:K05614]
131811339  SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613]
131815352  KCNJ10; ATP-sensitive inward rectifier potassium channel 10 [KO:K05003]
131823748  REST; RE1-silencing transcription factor isoform X1 [KO:K09222]
131835759  SIN3A; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
131837013  RCOR1; REST corepressor 1 [KO:K11829]
131809310  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
131833482  HDAC2; histone deacetylase 2 isoform X1 [KO:K06067] [EC:3.5.1.98]
131816293  POLR2A; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
131823726  POLR2B; DNA-directed RNA polymerase II subunit RPB2 [KO:K03010] [EC:2.7.7.6]
131818917  POLR2C; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
131826877  POLR2D; DNA-directed RNA polymerase II subunit RPB4 isoform X1 [KO:K03012]
131823182  POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1 [KO:K03013]
131825540  DNA-directed RNA polymerases I, II, and III subunit RPABC1-like [KO:K03013]
131839465  POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2 [KO:K03014]
131814132  POLR2G; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
131824785  POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
131817795  POLR2I; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
131818106  POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
131819178  POLR2J; DNA-directed RNA polymerase II subunit RPB11-a [KO:K03008]
131826551  POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
131823510  LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
131810728  BDNF; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355]
131839523  EP300; histone acetyltransferase p300 [KO:K04498] [EC:2.3.1.48]
131819828  CREBBP; CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
131840537  TAF4; transcription initiation factor TFIID subunit 4 isoform X1 [KO:K03129]
131811406  TAF4B; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
131833779  TBPL1; TATA box-binding protein-like 1 [KO:K03120]
131835358  TBPL2; TATA box-binding protein-like 2 [KO:K03120]
131833326  TBP; TATA-box-binding protein [KO:K03120]
131838694  SP1; transcription factor Sp1 [KO:K04684]
131827517  CREB1; cyclic AMP-responsive element-binding protein 1 isoform X1 [KO:K05870]
131811907  CREB3L1; cyclic AMP-responsive element-binding protein 3-like protein 1 [KO:K09048]
131812775  CREB3; cyclic AMP-responsive element-binding protein 3 isoform X1 [KO:K09048]
131814622  CREB3L4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
131823313  CREB3L3; cyclic AMP-responsive element-binding protein 3-like protein 3 [KO:K09048]
131829815  CREB3L2; cyclic AMP-responsive element-binding protein 3-like protein 2 isoform X1 [KO:K09048]
131829554  CREB5; cyclic AMP-responsive element-binding protein 5 isoform X1 [KO:K09047]
131825299  PPARGC1A; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X1 [KO:K07202]
131824235  PPARG; peroxisome proliferator-activated receptor gamma isoform X1 [KO:K08530]
131828949  TFAM; transcription factor A, mitochondrial [KO:K11830]
131829755  NRF1; nuclear respiratory factor 1 isoform X1 [KO:K11831]
131825303  SOD1; superoxide dismutase [Cu-Zn] [KO:K04565] [EC:1.15.1.1]
131833407  SOD2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
131836687  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
131823927  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
131831788  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
131809569  GPX7; glutathione peroxidase 7 isoform X1 [KO:K00432] [EC:1.11.1.9]
131832230  GPX8; probable glutathione peroxidase 8 isoform X1 [KO:K00432] [EC:1.11.1.9]
131832997  GPX6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
131833054  epididymal secretory glutathione peroxidase-like [KO:K00432] [EC:1.11.1.9]
131827532  UCP1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
131815809  TP53; cellular tumor antigen p53 isoform X1 [KO:K04451]
131817775  BAX; apoptosis regulator BAX [KO:K02159]
131818362  BBC3; bcl-2-binding component 3 [KO:K10132]
131838643  LOW QUALITY PROTEIN: NADH-ubiquinone oxidoreductase chain 5-like [KO:K03883] [EC:7.1.1.2]
131838648  NADH-ubiquinone oxidoreductase chain 6-like [KO:K03884] [EC:7.1.1.2]
131816609  NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
131810801  NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
131825420  NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
131821701  NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
131808139  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
131808426  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
131832156  NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946]
131818903  NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
131832350  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947]
131838568  NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948]
131829435  NDUFA4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
131829695  NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
131839559  NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 isoform X1 [KO:K03950]
131823416  NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
131812647  NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
131838421  NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
131827268  NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954]
131819982  NDUFAB1; acyl carrier protein, mitochondrial [KO:K03955]
131833597  acyl carrier protein, mitochondrial-like [KO:K03955]
131823366  NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
131839278  NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352]
131823813  NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
131825818  LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957]
131829846  NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958]
131830331  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958]
131827581  NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
131829831  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
131823076  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
131825074  NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
131824842  NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961]
131812517  NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
131823651  NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
131828542  NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964]
131826423  NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965]
131819903  NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
131821235  NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
131827534  NDUFS1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934] [EC:7.1.1.2]
131814323  NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
131812586  NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
131831733  NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
131809677  NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
131831547  NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939]
131823196  NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
131821226  NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial-like [KO:K03941] [EC:7.1.1.2]
131816678  NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
131827605  NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
131840274  NDUFC2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
131831512  SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234] [EC:1.3.5.1]
131809129  SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
131814529  SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236]
131835909  SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237]
131818257  cytochrome b-c1 complex subunit Rieske, mitochondrial [KO:K00411] [EC:7.1.1.8]
131826348  cytochrome c1, heme protein, mitochondrial [KO:K00413]
131823896  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
131819847  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
131828956  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415]
131809696  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
131826578  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
131821062  cytochrome b-c1 complex subunit 7-like [KO:K00417]
131829633  cytochrome b-c1 complex subunit 7-like [KO:K00417]
131808187  cytochrome b-c1 complex subunit 7-like [KO:K00417]
131809510  cytochrome b-c1 complex subunit 7-like [KO:K00417]
131832029  cytochrome b-c1 complex subunit 8 [KO:K00418]
131811280  cytochrome b-c1 complex subunit 9 isoform X1 [KO:K00419]
131823213  cytochrome b-c1 complex subunit 10 [KO:K00420]
131807891  cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263]
131817957  cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263]
131835769  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
131808567  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
131826632  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
131833910  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
131811812  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
131819690  cytochrome c oxidase subunit 6A2, mitochondrial isoform X1 [KO:K02266]
131825927  cytochrome c oxidase subunit 6A1, mitochondrial-like [KO:K02266]
131818583  cytochrome c oxidase subunit 6B2 [KO:K02267]
131818340  cytochrome c oxidase subunit 6B1 [KO:K02267]
131840480  cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270]
131833681  cytochrome c oxidase subunit 7A2, mitochondrial [KO:K02270]
131821253  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
131817997  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
131821722  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
131809285  cytochrome c oxidase subunit 7B, mitochondrial-like [KO:K02271]
131824398  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
131836628  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
131812224  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
131822093  cytochrome c oxidase subunit 7C, mitochondrial-like [KO:K02272]
131832432  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
131814508  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
131833172  cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273]
131811412  ATP5F1A; ATP synthase subunit alpha, mitochondrial [KO:K02132]
131819717  LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132]
131838591  ATP5F1B; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
131838082  ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
131829706  ATP synthase subunit gamma, mitochondrial [KO:K02136]
131823189  ATP5F1D; ATP synthase subunit delta, mitochondrial [KO:K02134]
131834304  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
131834699  ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
131807672  ATP5F1E; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
131808814  ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
131838683  ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128]
131827065  ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
131821176  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
131815524  ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128]
131808082  ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128]
131836058  ATP synthase subunit d, mitochondrial-like [KO:K02138]
131815877  ATP5PD; ATP synthase subunit d, mitochondrial [KO:K02138]
131825333  ATP5PO; ATP synthase subunit O, mitochondrial [KO:K02137]
131825280  ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131]
131836545  voltage-dependent anion-selective channel protein 1-like [KO:K05862]
131832037  VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
131828797  VDAC2; LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 2 [KO:K15040]
131820455  VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
131832988  voltage-dependent anion-selective channel protein 3-like [KO:K15041]
131820320  SLC25A4; ADP/ATP translocase 1 [KO:K05863]
131828020  SLC25A31; ADP/ATP translocase 4 [KO:K05863]
131821117  SLC25A5; ADP/ATP translocase 2 [KO:K05863]
131821316  SLC25A6; ADP/ATP translocase 3 [KO:K05863]
131828775  PPIF; peptidyl-prolyl cis-trans isomerase F, mitochondrial isoform X1 [KO:K09565] [EC:5.2.1.8]
131833963  cytochrome c-like [KO:K08738]
131834139  cytochrome c [KO:K08738]
131829521  cytochrome c [KO:K08738]
131839300  APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084]
131809520  CASP9; caspase-9 isoform X1 [KO:K04399] [EC:3.4.22.62]
131836468  PSMA6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
131819827  LOW QUALITY PROTEIN: proteasome subunit alpha type-2-like [KO:K02726] [EC:3.4.25.1]
131829141  PSMA2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
131835471  PSMA4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
131811262  PSMA8; proteasome subunit alpha-type 8 [KO:K02731] [EC:3.4.25.1]
131808524  PSMA7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
131809922  PSMA5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
131812899  proteasome subunit alpha type-1-like [KO:K02725] [EC:3.4.25.1]
131809603  PSMA1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
131832701  proteasome subunit alpha type-1-like isoform X1 [KO:K02725] [EC:3.4.25.1]
131836619  PSMA3; proteasome subunit alpha type-3 [KO:K02727] [EC:3.4.25.1]
131816177  PSMB6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
131812961  PSMB7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
131815758  PSMB3; proteasome subunit beta type-3 isoform X1 [KO:K02735] [EC:3.4.25.1]
131809673  PSMB2; proteasome subunit beta type-2 [KO:K02734] [EC:3.4.25.1]
131837205  PSMB5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
131812267  proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
131840119  proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
131833327  PSMB1; proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
131810591  PSMB4; proteasome subunit beta type-4 isoform X1 [KO:K02736] [EC:3.4.25.1]
131811027  26S proteasome regulatory subunit 7-like [KO:K03061]
131829278  PSMC2; 26S proteasome regulatory subunit 7 [KO:K03061]
131836774  PSMC1; 26S proteasome regulatory subunit 4 [KO:K03062]
131815002  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
131818300  PSMC4; 26S proteasome regulatory subunit 6B [KO:K03063]
131836571  PSMC6; 26S proteasome regulatory subunit 10B isoform X1 [KO:K03064]
131812443  PSMC3; 26S proteasome regulatory subunit 6A [KO:K03065]
131823718  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 6A-like [KO:K03065]
131816915  PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
131824790  PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
131827346  PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
131816097  PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
131811370  PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693]
131816183  PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
131816024  PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036]
131824095  PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037]
131818093  PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
131818523  PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039]
131808866  PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029]
131827172  PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030]
131817762  PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
131808593  ADRM1; proteasomal ubiquitin receptor ADRM1 [KO:K06691]
131829409  SEM1; 26S proteasome complex subunit SEM1 [KO:K10881]
131817505  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
131816957  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 isoform X1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
131812685  TRAF2; TNF receptor-associated factor 2 isoform X1 [KO:K03173] [EC:2.3.2.27]
131833762  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
131823426  MAP2K7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
131810411  MTOR; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
131811225  ULK1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
131817041  serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
131838739  ATG101; autophagy-related protein 101 [KO:K19730]
131812082  ATG13; autophagy-related protein 13 isoform X1 [KO:K08331]
131826840  RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
131812046  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
131815214  BECN2; beclin-2 [KO:K08334]
131816325  BECN1; beclin-1 isoform X1 [KO:K08334]
131824530  PIK3R4; phosphoinositide 3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
131836601  ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889]
131828915  NRBF2; nuclear receptor-binding factor 2 [KO:K21246]
131811092  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 isoform X1 [KO:K00914] [EC:2.7.1.137]
131836963  ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
131815013  ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906]
131819416  WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908]
131816133  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
mlk00190  Oxidative phosphorylation
mlk03020  RNA polymerase
mlk03022  Basal transcription factors
mlk03050  Proteasome
mlk04020  Calcium signaling pathway
mlk04115  p53 signaling pathway
mlk04140  Autophagy - animal
mlk04141  Protein processing in endoplasmic reticulum
mlk04144  Endocytosis
mlk04210  Apoptosis
mlk04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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