KEGG   PATHWAY: mmma05016
Entry
mmma05016                   Pathway                                
Name
Huntington disease - Marmota marmota marmota (Alpine marmot)
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
mmma05016  Huntington disease
mmma05016

Organism
Marmota marmota marmota (Alpine marmot) [GN:mmma]
Gene
107147794  Clta; clathrin light chain A isoform X4 [KO:K04644]
107144705  Cltb; clathrin light chain B isoform X2 [KO:K04645]
107150387  Cltc; clathrin heavy chain 1 isoform X2 [KO:K04646]
107154713  Cltcl1; clathrin heavy chain 2 isoform X2 [KO:K04646]
107147046  Hip1; huntingtin-interacting protein 1 isoform X1 [KO:K04559]
107151232  Ap2a1; AP-2 complex subunit alpha-1 isoform X1 [KO:K11824]
107157078  Ap2a2; AP-2 complex subunit alpha-2 isoform X3 [KO:K11824]
107150332  Ap2b1; AP-2 complex subunit beta isoform X1 [KO:K11825]
107154317  Ap2m1; AP-2 complex subunit mu isoform X1 [KO:K11826]
107151322  Ap2s1; AP-2 complex subunit sigma [KO:K11827]
107139096  Htt; huntingtin isoform X1 [KO:K04533]
107154215  Dctn1; dynactin subunit 1 isoform X11 [KO:K04648]
107140131  Dctn2; dynactin subunit 2 isoform X3 [KO:K10424]
107147836  Dctn3; dynactin subunit 3 isoform X1 [KO:K10425]
107142840  Dctn4; dynactin subunit 4 isoform X2 [KO:K10426]
107146869  Dctn5; dynactin subunit 5 isoform X1 [KO:K10427]
107156464  dynactin subunit 6-like [KO:K10428]
107155357  Dctn6; dynactin subunit 6 isoform X1 [KO:K10428]
107149838  Actr1a; alpha-centractin [KO:K16575]
107154121  Actr1b; beta-centractin [KO:K16575]
107138311  Actr10; actin-related protein 10 isoform X1 [KO:K16576]
107150588  Hap1; huntingtin-associated protein 1 isoform X1 [KO:K04647]
107149705  Dnah1; dynein axonemal heavy chain 1 isoform X2 [KO:K10408]
107143624  Dnah8; dynein axonemal heavy chain 8 [KO:K10408]
107158049  Dnah9; dynein axonemal heavy chain 9 isoform X1 [KO:K10408]
107138353  Dnah7; dynein axonemal heavy chain 7 isoform X1 [KO:K10408]
107140282  Dnah14; dynein axonemal heavy chain 14 [KO:K10408]
107146320  Dnah5; dynein axonemal heavy chain 5 isoform X1 [KO:K10408]
107154254  Dnah6; dynein axonemal heavy chain 6 isoform X1 [KO:K10408]
107156340  Dnah10; dynein axonemal heavy chain 10 isoform X1 [KO:K10408]
107147153  Dnah3; dynein axonemal heavy chain 3 isoform X1 [KO:K10408]
107157847  Dnah17; dynein axonemal heavy chain 17 [KO:K10408]
107158008  Dnah2; dynein axonemal heavy chain 2 isoform X1 [KO:K10408]
107152632  Dnah11; dynein axonemal heavy chain 11 isoform X1 [KO:K10408]
107149695  Dnah12; dynein axonemal heavy chain 12 [KO:K10408]
107141508  Dnali1; axonemal dynein light intermediate polypeptide 1 [KO:K10410]
107147649  Dnai1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409]
107157219  Dnai2; dynein axonemal intermediate chain 2 isoform X2 [KO:K11143]
107148964  Dnal1; dynein axonemal light chain 1 [KO:K10411]
107139854  Dnal4; dynein axonemal light chain 4 [KO:K10412]
107138719  tubulin alpha-4A chain [KO:K07374]
107138722  tubulin alpha-1D chain isoform X1 [KO:K07374]
107134536  tubulin alpha-1B chain-like [KO:K07374]
107140681  tubulin alpha-8 chain isoform X1 [KO:K07374]
107141892  tubulin alpha-1C chain-like isoform X1 [KO:K07374]
107148600  Tuba1a; tubulin alpha-1A chain [KO:K07374]
107148601  tubulin alpha-1C chain [KO:K07374]
107150280  Tubal3; tubulin alpha chain-like 3 [KO:K07374]
107154571  tubulin alpha-8 chain-like [KO:K07374]
107154692  tubulin alpha-3 chain [KO:K07374]
107154696  tubulin alpha-3 chain [KO:K07374]
107155040  Tuba8; tubulin alpha-8 chain isoform X1 [KO:K07374]
107149203  Tubb2a; tubulin beta-2A chain [KO:K07375]
107149205  tubulin beta-2B chain [KO:K07375]
107137659  Tubb1; tubulin beta-1 chain isoform X1 [KO:K07375]
107150800  Tubb3; tubulin beta-3 chain [KO:K07375]
107134810  uncharacterized protein D806_0078-like [KO:K07375]
107159219  Tubb4b; tubulin beta-4B chain [KO:K07375]
107159459  tubulin beta-5 chain [KO:K07375]
107160457  Tubb; tubulin beta chain [KO:K07375]
107146111  tubulin beta chain-like [KO:K07375]
107146249  tubulin beta-2A chain-like isoform X1 [KO:K07375]
107157327  Tubb4a; tubulin beta-4A chain [KO:K07375]
107159793  Mapk9; mitogen-activated protein kinase 9 isoform X1 [KO:K04440] [EC:2.7.11.24]
107153372  Mapk8; mitogen-activated protein kinase 8 isoform X1 [KO:K04440] [EC:2.7.11.24]
107153644  Mapk10; mitogen-activated protein kinase 10 isoform X1 [KO:K04440] [EC:2.7.11.24]
107144171  LOW QUALITY PROTEIN: mitogen-activated protein kinase 8-like [KO:K04440] [EC:2.7.11.24]
107140127  Kif5a; kinesin heavy chain isoform X2 [KO:K10396]
107140244  Kif5b; kinesin-1 heavy chain [KO:K10396]
107136617  Kif5c; LOW QUALITY PROTEIN: kinesin heavy chain [KO:K10396]
107151611  Klc3; kinesin light chain 3 [KO:K10407]
107143933  Klc4; kinesin light chain 4 isoform X1 [KO:K10407]
107150940  Klc1; kinesin light chain 1 isoform X1 [KO:K10407]
107156961  Klc2; kinesin light chain 2 isoform X1 [KO:K10407]
107136338  Ift57; intraflagellar transport protein 57 homolog [KO:K04638]
107138607  Casp8; caspase-8 isoform X1 [KO:K04398] [EC:3.4.22.61]
107142678  Casp3; caspase-3 isoform X1 [KO:K02187] [EC:3.4.22.56]
107158031  caspase-3-like [KO:K02187] [EC:3.4.22.56]
107135439  Grm5; metabotropic glutamate receptor 5 isoform X1 [KO:K04604]
107148216  guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634]
107135966  Plcb3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858] [EC:3.1.4.11]
107138232  Plcb2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858] [EC:3.1.4.11]
107145524  1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X3 [KO:K05858] [EC:3.1.4.11]
107145453  Plcb1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858] [EC:3.1.4.11]
107147036  Stx1a; syntaxin-1A [KO:K04560]
107159103  Cacna1b; LOW QUALITY PROTEIN: voltage-dependent N-type calcium channel subunit alpha-1B [KO:K04849]
107159190  Grin1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208]
107152685  Grin2b; glutamate receptor ionotropic, NMDA 2B [KO:K05210]
107150041  Itpr1; inositol 1,4,5-trisphosphate receptor type 1 isoform X11 [KO:K04958]
107157969  Dlg4; disks large homolog 4 isoform X1 [KO:K11828]
107148089  Gria1; glutamate receptor 1 isoform X1 [KO:K05197]
107141203  Gria2; glutamate receptor 2 isoform X4 [KO:K05198]
107153037  Gria3; glutamate receptor 3 [KO:K05199]
107145895  Gria4; glutamate receptor 4 isoform X2 [KO:K05200]
107138178  Tgm2; protein-glutamine gamma-glutamyltransferase 2 [KO:K05625] [EC:2.3.2.13]
107151115  Slc1a3; excitatory amino acid transporter 1 [KO:K05614]
107135755  Slc1a2; excitatory amino acid transporter 2 isoform X3 [KO:K05613]
107147512  Kcnj10; ATP-sensitive inward rectifier potassium channel 10 isoform X2 [KO:K05003]
107139256  Rest; RE1-silencing transcription factor [KO:K09222]
107137930  Sin3a; paired amphipathic helix protein Sin3a isoform X1 [KO:K11644]
107144848  Rcor1; REST corepressor 1 isoform X1 [KO:K11829]
107142001  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
107154686  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
107157993  Polr2a; DNA-directed RNA polymerase II subunit RPB1 [KO:K03006] [EC:2.7.7.6]
107149608  LOW QUALITY PROTEIN: DNA-directed RNA polymerase II subunit RPB2-like [KO:K03010] [EC:2.7.7.6]
107139257  Polr2b; DNA-directed RNA polymerase II subunit RPB2 isoform X1 [KO:K03010] [EC:2.7.7.6]
107144829  Polr2c; DNA-directed RNA polymerase II subunit RPB3 [KO:K03011]
107136797  Polr2d; DNA-directed RNA polymerase II subunit RPB4 isoform X2 [KO:K03012]
107149293  DNA-directed RNA polymerase II subunit RPB4-like [KO:K03012]
107157238  Polr2e; DNA-directed RNA polymerases I, II, and III subunit RPABC1 isoform X1 [KO:K03013]
107139869  Polr2f; DNA-directed RNA polymerases I, II, and III subunit RPABC2 isoform X1 [KO:K03014]
107135931  Polr2g; DNA-directed RNA polymerase II subunit RPB7 [KO:K03015]
107145597  DNA-directed RNA polymerases I, II, and III subunit RPABC3-like [KO:K03016]
107154327  Polr2h; DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
107151820  Polr2i; DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
107142300  Polr2l; LOW QUALITY PROTEIN: DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
107142833  Polr2k; DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
107142368  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
107154336  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
107144950  DNA-directed RNA polymerases I, II, and III subunit RPABC4-like [KO:K03009]
107134338  Bdnf; brain-derived neurotrophic factor isoform X1 [KO:K04355]
107146666  CREB-binding protein isoform X1 [KO:K04498] [EC:2.3.1.48]
107155853  Ep300; histone acetyltransferase p300 isoform X1 [KO:K04498] [EC:2.3.1.48]
107154107  Taf4; transcription initiation factor TFIID subunit 4 [KO:K03129]
107139568  Taf4b; transcription initiation factor TFIID subunit 4B isoform X1 [KO:K03129]
107138299  Tbpl2; TATA box-binding protein-like 2 isoform X1 [KO:K03120]
107153083  TATA-box-binding protein [KO:K03120]
107155540  Tbpl1; TATA box-binding protein-like 1 [KO:K03120]
107148811  Sp1; transcription factor Sp1 isoform X1 [KO:K04684]
107138645  Creb1; cyclic AMP-responsive element-binding protein 1 isoform X2 [KO:K05870]
107147808  Creb3; cyclic AMP-responsive element-binding protein 3 [KO:K09048]
107135791  Creb3l1; cyclic AMP-responsive element-binding protein 3-like protein 1 isoform X3 [KO:K09048]
107149021  Creb3l2; cyclic AMP-responsive element-binding protein 3-like protein 2 [KO:K09048]
107156760  Creb3l4; cyclic AMP-responsive element-binding protein 3-like protein 4 isoform X1 [KO:K09048]
107146134  Creb5; cyclic AMP-responsive element-binding protein 5 isoform X2 [KO:K09047]
107139157  Ppargc1a; peroxisome proliferator-activated receptor gamma coactivator 1-alpha isoform X3 [KO:K07202]
107150087  Pparg; peroxisome proliferator-activated receptor gamma isoform X2 [KO:K08530]
107158213  Tfam; transcription factor A, mitochondrial [KO:K11830]
107149058  Nrf1; nuclear respiratory factor 1 isoform X2 [KO:K11831]
107136431  Sod1; superoxide dismutase [Cu-Zn] isoform X2 [KO:K04565] [EC:1.15.1.1]
107149756  superoxide dismutase [Cu-Zn]-like [KO:K04565] [EC:1.15.1.1]
107153122  Sod2; superoxide dismutase [Mn], mitochondrial [KO:K04564] [EC:1.15.1.1]
107143484  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
107148105  Gpx3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
107141762  Gpx7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
107151887  Gpx8; probable glutathione peroxidase 8 isoform X2 [KO:K00432] [EC:1.11.1.9]
107160503  Gpx5; epididymal secretory glutathione peroxidase [KO:K00432] [EC:1.11.1.9]
107160504  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
107146162  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
107141188  Ucp1; mitochondrial brown fat uncoupling protein 1 [KO:K08769]
107158007  Tp53; cellular tumor antigen p53 [KO:K04451]
107151268  Bax; apoptosis regulator BAX isoform X1 [KO:K02159]
107156880  Ndufv1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial [KO:K03942] [EC:7.1.1.2]
107139635  Ndufv2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial [KO:K03943] [EC:7.1.1.2]
107136511  Ndufv3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944]
107152995  Ndufa1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945]
107146270  NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945]
107141755  Ndufa2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946]
107158928  Ndufa3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947]
107144474  Ndufa4; cytochrome c oxidase subunit NDUFA4 [KO:K03948]
107140143  Ndufa4l2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 isoform X1 [KO:K03948]
107144505  Ndufa5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949]
107155829  Ndufa6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950]
107157374  Ndufa7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951]
107152121  Ndufa8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952]
107154983  Ndufa9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953]
107138852  Ndufa10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954]
107146550  Ndufab1; acyl carrier protein, mitochondrial [KO:K03955]
107157461  Ndufa11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956]
107140004  Ndufa12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352]
107145383  Ndufa13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353]
107144262  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
107149970  Ndufb1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957]
107149001  Ndufb2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958]
107138601  Ndufb3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959]
107151079  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3-like [KO:K03959]
107143262  Ndufb4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960]
107152489  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960]
107149458  Ndufb5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961]
107147859  Ndufb6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962]
107145272  Ndufb7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963]
107149806  Ndufb8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964]
107142910  Ndufb9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965]
107146729  Ndufb10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966]
107138463  Ndufb11; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
107135241  NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351]
107138634  Ndufs1; NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial isoform X1 [KO:K03934] [EC:7.1.1.2]
107147471  Ndufs2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935] [EC:7.1.1.2]
107135819  Ndufs3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936] [EC:7.1.1.2]
107151897  Ndufs4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937]
107144351  NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938]
107141924  Ndufs5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938]
107146364  Ndufs6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939]
107157247  Ndufs7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940] [EC:7.1.1.2]
107157021  Ndufs8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941] [EC:7.1.1.2]
107136771  Ndufc1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967]
107135475  Ndufc2; NADH dehydrogenase [ubiquinone] 1 subunit C2 [KO:K03968]
107146296  Sdha; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234] [EC:1.3.5.1]
107141579  Sdhb; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235] [EC:1.3.5.1]
107147466  Sdhc; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236]
107145856  Sdhd; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237]
107150640  succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
107151812  LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237]
107149921  LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit Rieske, mitochondrial-like [KO:K00411] [EC:7.1.1.8]
107143017  cytochrome c1, heme protein, mitochondrial [KO:K00413]
107143515  cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
107146855  cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
107141806  cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416]
107142519  cytochrome b-c1 complex subunit 7 isoform X1 [KO:K00417]
107148123  cytochrome b-c1 complex subunit 8 [KO:K00418]
107154580  cytochrome b-c1 complex subunit 9 [KO:K00419]
107157256  cytochrome b-c1 complex subunit 10 [KO:K00420]
107150843  cytochrome c oxidase subunit 4 isoform 1, mitochondrial isoform X2 [KO:K02263]
107134718  cytochrome c oxidase subunit 4 isoform 2, mitochondrial-like [KO:K02263]
107159065  cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263]
107137948  cytochrome c oxidase subunit 5A, mitochondrial [KO:K02264]
107154122  cytochrome c oxidase subunit 5B, mitochondrial [KO:K02265]
107148749  LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5B, mitochondrial-like [KO:K02265]
107146984  cytochrome c oxidase subunit 6A2, mitochondrial [KO:K02266]
107158712  cytochrome c oxidase subunit 6A1, mitochondrial [KO:K02266]
107147180  cytochrome c oxidase subunit 6B1-like [KO:K02267]
107158929  cytochrome c oxidase subunit 6B2 [KO:K02267]
107151834  cytochrome c oxidase subunit 6B1 [KO:K02267]
107142829  cytochrome c oxidase subunit 6C [KO:K02268]
107137328  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
107137972  cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270]
107149546  cytochrome c oxidase subunit 7A-related protein, mitochondrial-like [KO:K02270]
107151835  cytochrome c oxidase subunit 7A1, mitochondrial [KO:K02270]
107153341  cytochrome c oxidase subunit 7A2, mitochondrial isoform X2 [KO:K02270]
107134143  cytochrome c oxidase subunit 7A2, mitochondrial-like [KO:K02270]
107160422  cytochrome c oxidase subunit 7A-related protein, mitochondrial-like [KO:K02270]
107138951  cytochrome c oxidase subunit 7B2, mitochondrial [KO:K02271]
107159289  cytochrome c oxidase subunit 7B, mitochondrial [KO:K02271]
107152336  cytochrome c oxidase subunit 7C, mitochondrial [KO:K02272]
107135954  cytochrome c oxidase subunit 8A, mitochondrial [KO:K02273]
107157121  cytochrome c oxidase subunit 8B, mitochondrial-like [KO:K02273]
107139527  Atp5f1a; ATP synthase subunit alpha, mitochondrial [KO:K02132]
107140159  Atp5f1b; ATP synthase subunit beta, mitochondrial [KO:K02133] [EC:7.1.2.2]
107150226  Atp5f1c; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136]
107157405  Atp5f1d; ATP synthase subunit delta, mitochondrial [KO:K02134]
107148314  LOW QUALITY PROTEIN: ATP synthase subunit epsilon, mitochondrial-like [KO:K02135]
107138216  Atp5f1e; ATP synthase subunit epsilon, mitochondrial [KO:K02135]
107156135  Atp5pb; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127]
107136940  Atp5mc3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128]
107150479  Atp5mc1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X2 [KO:K02128]
107148754  Atp5mc2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
107152831  LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
107147554  ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128]
107143163  ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128]
125613532  ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128]
107157798  Atp5pd; ATP synthase subunit d, mitochondrial [KO:K02138]
107136450  Atp5po; ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137]
107136415  Atp5pf; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131]
107146908  LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862]
107148006  Vdac1; voltage-dependent anion-selective channel protein 1 [KO:K05862]
107159648  Vdac3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041]
107136662  Slc25a31; ADP/ATP translocase 4 [KO:K05863]
107142667  Slc25a4; ADP/ATP translocase 1 [KO:K05863]
107153003  Slc25a5; ADP/ATP translocase 2 [KO:K05863]
107158131  Ppif; peptidyl-prolyl cis-trans isomerase F, mitochondrial [KO:K09565] [EC:5.2.1.8]
107146118  cytochrome c, somatic [KO:K08738]
125618140  LOW QUALITY PROTEIN: cytochrome c, testis-specific-like [KO:K08738]
107161017  cytochrome c, somatic [KO:K08738]
107149067  cytochrome c, somatic-like [KO:K08738]
107135575  cytochrome c, somatic-like [KO:K08738]
107151095  cytochrome c, somatic-like [KO:K08738]
107136200  cytochrome c, somatic-like [KO:K08738]
107139684  Apaf1; LOW QUALITY PROTEIN: apoptotic protease-activating factor 1 [KO:K02084]
107142215  Casp9; caspase-9 [KO:K04399] [EC:3.4.22.62]
107147107  Psma6; proteasome subunit alpha type-6 [KO:K02730] [EC:3.4.25.1]
107146203  Psma2; proteasome subunit alpha type-2 [KO:K02726] [EC:3.4.25.1]
107137908  Psma4; proteasome subunit alpha type-4 [KO:K02728] [EC:3.4.25.1]
107136437  Psma7; proteasome subunit alpha type-7 [KO:K02731] [EC:3.4.25.1]
107139410  Psma8; proteasome subunit alpha-type 8 isoform X2 [KO:K02731] [EC:3.4.25.1]
107134499  proteasome subunit alpha type-7-like [KO:K02731] [EC:3.4.25.1]
107158620  Psma5; proteasome subunit alpha type-5 isoform X1 [KO:K02729] [EC:3.4.25.1]
107135668  Psma1; proteasome subunit alpha type-1 isoform X1 [KO:K02725] [EC:3.4.25.1]
107138315  Psma3; proteasome subunit alpha type-3 isoform X1 [KO:K02727] [EC:3.4.25.1]
107152020  proteasome subunit beta type-6-like [KO:K02738] [EC:3.4.25.1]
107159412  Psmb6; proteasome subunit beta type-6 [KO:K02738] [EC:3.4.25.1]
107152100  Psmb7; proteasome subunit beta type-7 [KO:K02739] [EC:3.4.25.1]
107160021  Psmb3; proteasome subunit beta type-3 [KO:K02735] [EC:3.4.25.1]
107141960  Psmb2; proteasome subunit beta type-2 isoform X1 [KO:K02734] [EC:3.4.25.1]
107148113  Psmb5; proteasome subunit beta type-5 [KO:K02737] [EC:3.4.25.1]
107137384  proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
107153084  Psmb1; LOW QUALITY PROTEIN: proteasome subunit beta type-1 [KO:K02732] [EC:3.4.25.1]
107156804  Psmb4; proteasome subunit beta type-4 [KO:K02736] [EC:3.4.25.1]
107152391  26S proteasome regulatory subunit 7-like [KO:K03061]
107149837  Psmc1; 26S proteasome regulatory subunit 4 [KO:K03062]
107142327  LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 4-like [KO:K03062]
107151465  Psmc4; 26S proteasome regulatory subunit 6B [KO:K03063]
107138279  Psmc6; 26S proteasome regulatory subunit 10B [KO:K03064]
107135815  Psmc3; 26S proteasome regulatory subunit 6A isoform X1 [KO:K03065]
107158336  Psmc5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066]
107154323  Psmd2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028]
107138782  Psmd1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032]
107150628  Psmd3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033]
107156410  Psmd9; 26S proteasome non-ATPase regulatory subunit 9 isoform X3 [KO:K06693]
107158356  Psmd12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035]
107155158  Psmd11; 26S proteasome non-ATPase regulatory subunit 11 isoform X1 [KO:K03036]
107149672  Psmd6; 26S proteasome non-ATPase regulatory subunit 6 isoform X2 [KO:K03037]
107150939  Psmd7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038]
107157120  Psmd13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039]
107156810  Psmd4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029]
107136883  Psmd14; 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030]
107142356  26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
107151495  Psmd8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031]
107136373  Adrm1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691]
125611362  Sem1; 26S proteasome complex subunit SEM1 [KO:K10881]
107151641  Map3k10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
107158342  Ern1; serine/threonine-protein kinase/endoribonuclease IRE1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
107159171  Traf2; TNF receptor-associated factor 2 isoform X2 [KO:K03173] [EC:2.3.2.27]
107155636  Map3k5; mitogen-activated protein kinase kinase kinase 5 isoform X1 [KO:K04426] [EC:2.7.11.25]
107157355  Map2k7; dual specificity mitogen-activated protein kinase kinase 7 isoform X1 [KO:K04431] [EC:2.7.12.2]
107155502  Mtor; serine/threonine-protein kinase mTOR [KO:K07203] [EC:2.7.11.1]
107159067  Ulk1; serine/threonine-protein kinase ULK1 isoform X1 [KO:K21357] [EC:2.7.11.1]
107160172  Ulk2; serine/threonine-protein kinase ULK2 isoform X1 [KO:K08269] [EC:2.7.11.1]
107148648  Atg101; autophagy-related protein 101 [KO:K19730]
107135797  Atg13; autophagy-related protein 13 isoform X1 [KO:K08331]
107142384  Rb1cc1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589]
107135795  Ambra1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985]
107150548  Becn1; beclin-1 isoform X1 [KO:K08334]
107140265  Becn2; beclin-2 [KO:K08334]
107143405  Pik3r4; phosphoinositide 3-kinase regulatory subunit 4 isoform X2 [KO:K08333] [EC:2.7.11.1]
107138297  Atg14; beclin 1-associated autophagy-related key regulator [KO:K17889]
107158234  Nrbf2; nuclear receptor-binding factor 2 isoform X1 [KO:K21246]
107139532  Pik3c3; LOW QUALITY PROTEIN: phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
107150385  Atg2b; autophagy-related protein 2 homolog B isoform X1 [KO:K17906]
107135989  Atg2a; autophagy-related protein 2 homolog A [KO:K17906]
107158368  Wipi1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908]
107155980  Wipi2; WD repeat domain phosphoinositide-interacting protein 2 isoform X3 [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
mmma00190  Oxidative phosphorylation
mmma03020  RNA polymerase
mmma03022  Basal transcription factors
mmma03050  Proteasome
mmma04020  Calcium signaling pathway
mmma04115  p53 signaling pathway
mmma04140  Autophagy - animal
mmma04141  Protein processing in endoplasmic reticulum
mmma04144  Endocytosis
mmma04210  Apoptosis
mmma04724  Glutamatergic synapse
KO pathway
ko05016   
LinkDB

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