KEGG   PATHWAY: mdo05016
Entry
mdo05016                    Pathway                                
Name
Huntington disease - Monodelphis domestica (gray short-tailed opossum)
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
mdo05016  Huntington disease
mdo05016

Disease
H00059  Huntington disease
Organism
Monodelphis domestica (gray short-tailed opossum) [GN:mdo]
Gene
100009766  IFT57; intraflagellar transport 57 [KO:K04638]
100009860  SLC1A2; solute carrier family 1 member 2 [KO:K05613]
100009917  GRM5; glutamate metabotropic receptor 5 [KO:K04604]
100010238  TUBA4A; tubulin alpha-4A chain [KO:K07374]
100010347  TUBA1B; tubulin alpha 1b [KO:K07374]
100010363  SLC25A6; solute carrier family 25 member 6 [KO:K05863]
100010436  TUBA1A; tubulin alpha-1 chain [KO:K07374]
100010481  [KO:K07374]
100010487  [KO:K05863]
100010541  NDUFB8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
100010612  BECN1; beclin 1 [KO:K08334]
100010620  [KO:K08738]
100010639  SLC25A31; solute carrier family 25 member 31 [KO:K05863]
100010650  [KO:K02267]
100010697  POLR2G; RNA polymerase II subunit G [KO:K03015]
100010713  CLTC; clathrin heavy chain [KO:K04646]
100010718  TAF4B; TATA-box binding protein associated factor 4b [KO:K03129]
100010801  PSMA8; proteasome subunit alpha 8 [KO:K02731] [EC:3.4.25.1]
100010999  KLC4; kinesin light chain 4 [KO:K10407]
100011101  PSMA6; proteasome subunit alpha 6 [KO:K02730] [EC:3.4.25.1]
100011149  WIPI2; WD repeat domain, phosphoinositide interacting 2 [KO:K17908]
100011263  PPARGC1A; PPARG coactivator 1 alpha [KO:K07202]
100011310  PSMD6; proteasome 26S subunit, non-ATPase 6 [KO:K03037]
100011397  PIK3C3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
100011414  ATP5C1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136]
100011523  [KO:K02272]
100011605  GNAQ; G protein subunit alpha q [KO:K04634]
100011759  ATG13; autophagy related 13 [KO:K08331]
100011981  AP2M1; adaptor related protein complex 2 mu 1 subunit [KO:K11826]
100011987  SLC25A4; solute carrier family 25 member 4 [KO:K05863]
100012012  NDUFB10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
100012168  NDUFA12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
100012197  POLR2B; RNA polymerase II subunit B [KO:K03010] [EC:2.7.7.6]
100012255  NDUFA13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
100012461  VDAC2; voltage dependent anion channel 2 [KO:K15040]
100012494  [KO:K02266]
100012527  [KO:K00411] [EC:7.1.1.8]
100012618  [KO:K02271]
100013047  CLTA; clathrin light chain A [KO:K04644]
100013051  APAF1; apoptotic peptidase activating factor 1 [KO:K02084]
100013066  PSMD8; proteasome 26S subunit, non-ATPase 8 [KO:K03031]
100013204  DNAL4; dynein axonemal light chain 4 [KO:K10412]
100013297  [KO:K02267]
100013305  ITPR1; inositol 1,4,5-trisphosphate receptor type 1 [KO:K04958]
100013423  UQCR11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
100013434  NDUFA5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
100013478  KIF5C; kinesin family member 5C [KO:K10396]
100013483  PSMA2; proteasome subunit alpha 2 [KO:K02726] [EC:3.4.25.1]
100013786  NDUFB5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
100013836  PSMC2; proteasome 26S subunit, ATPase 2 [KO:K03061]
100013866  PSMC6; proteasome 26S subunit, ATPase 6 [KO:K03064]
100013876  CREBBP; CREB binding protein [KO:K04498] [EC:2.3.1.48]
100013916  POLR2H; RNA polymerase II subunit H [KO:K03016]
100013993  NDUFV1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2]
100014040  TUBB6; tubulin beta 6 class V [KO:K07375]
100014049  CREB1; cAMP responsive element binding protein 1 [KO:K05870]
100014124  ATP5A1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132]
100014201  POLR2I; RNA polymerase II subunit I [KO:K03017]
100014350  ATP5B; ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide [KO:K02133] [EC:7.1.2.2]
100014533  SLC1A3; solute carrier family 1 member 3 [KO:K05614]
100014702  PPIF; peptidylprolyl isomerase F [KO:K09565] [EC:5.2.1.8]
100014728  POLR2A; RNA polymerase II subunit A [KO:K03006] [EC:2.7.7.6]
100014846  PSMD1; proteasome 26S subunit, non-ATPase 1 [KO:K03032]
100014869  VDAC1; voltage dependent anion channel 1 [KO:K05862]
100014941  PSMC4; proteasome 26S subunit, ATPase 4 [KO:K03063]
100014981  [KO:K02273]
100014993  SOD1; superoxide dismutase 1 [KO:K04565] [EC:1.15.1.1]
100015046  CREB3L1; cAMP responsive element binding protein 3 like 1 [KO:K09048]
100015047  NDUFAB1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
100015159  DNAH2; dynein axonemal heavy chain 2 [KO:K10408]
100015203  [KO:K07374]
100015315  HTT; huntingtin [KO:K04533]
100015411  NRF1; nuclear respiratory factor 1 [KO:K11831]
100015431  [KO:K17985]
100015558  PSMB7; proteasome subunit beta 7 [KO:K02739] [EC:3.4.25.1]
100015662  PSMD14; proteasome 26S subunit, non-ATPase 14 [KO:K03030]
100015745  PSMD3; proteasome 26S subunit, non-ATPase 3 [KO:K03033]
100015788  [KO:K00418]
100015794  POLR2E; RNA polymerase II subunit E [KO:K03013]
100015850  NDUFS6; NADH:ubiquinone oxidoreductase subunit S6 [KO:K03939]
100016000  REST; RE1 silencing transcription factor [KO:K09222]
100016103  NDUFB1; NADH:ubiquinone oxidoreductase subunit B1 [KO:K03957]
100016249  POLR2C; RNA polymerase II subunit C [KO:K03011]
100016464  [KO:K02734] [EC:3.4.25.1]
100016466  NDUFV2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2]
100016535  NDUFA8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
100016643  [KO:K02270]
100016655  DLG4; discs large MAGUK scaffold protein 4 [KO:K11828]
100016761  PSMC3; proteasome 26S subunit, ATPase 3 [KO:K03065]
100016818  NRBF2; nuclear receptor binding factor 2 [KO:K21246]
100016881  KLC1; kinesin light chain 1 [KO:K10407]
100016887  GPX8; glutathione peroxidase 8 (putative) [KO:K00432] [EC:1.11.1.9]
100016907  NDUFS3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2]
100017025  CASP18; caspase-18 [KO:K04398] [EC:3.4.22.61]
100017188  NDUFS1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2]
100017191  DNAH11; dynein axonemal heavy chain 11 [KO:K10408]
100017208  CREB3L2; cAMP responsive element binding protein 3 like 2 [KO:K09048]
100017209  POLR2F; RNA polymerase II subunit F [KO:K03014]
100017243  TRAF2; TNF receptor associated factor 2 [KO:K03173] [EC:2.3.2.27]
100017261  PSMB6; proteasome subunit beta 6 [KO:K02738] [EC:3.4.25.1]
100017266  NDUFB4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
100017355  GRIN2B; glutamate ionotropic receptor NMDA type subunit 2B [KO:K05210]
100017431  RCOR1; REST corepressor 1 [KO:K11829]
100017495  MAP3K10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
100017556  DCTN1; dynactin subunit 1 [KO:K04648]
100017630  [KO:K00413]
100017688  NDUFA7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
100017767  POLR2J; RNA polymerase II subunit J [KO:K03008]
100017800  AP2B1; adaptor related protein complex 2 beta 1 subunit [KO:K11825]
100018067  TUBB4B; tubulin beta 4B class IVb [KO:K07375]
100018068  [KO:K02263]
100018098  NDUFA6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
100018292  HIP1; huntingtin interacting protein 1 [KO:K04559]
100018337  [KO:K05863]
100018383  PSMB3; proteasome subunit beta 3 [KO:K02735] [EC:3.4.25.1]
100018385  PSMB4; proteasome subunit beta 4 [KO:K02736] [EC:3.4.25.1]
100018501  PSMC1; proteasome 26S subunit, ATPase 1 [KO:K03062]
100018504  GPX7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
100018533  POLR2D; RNA polymerase II subunit D [KO:K03012]
100018564  ATP5G1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C1 (subunit 9) [KO:K02128]
100018566  PSMD11; proteasome 26S subunit, non-ATPase 11 [KO:K03036]
100018937  ATG2B; autophagy related 2B [KO:K17906]
100018948  ATP5G3; ATP synthase, H+ transporting, mitochondrial Fo complex subunit C3 (subunit 9) [KO:K02128]
100019378  NDUFA9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
100019570  DCTN5; dynactin subunit 5 [KO:K10427]
100019912  NDUFC1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
100019961  [KO:K02267]
100019989  ULK2; unc-51 like autophagy activating kinase 2 [KO:K08269] [EC:2.7.11.1]
100020067  CREB3L4; cAMP responsive element binding protein 3 like 4 [KO:K09048]
100020164  DNAH5; dynein axonemal heavy chain 5 [KO:K10408]
100020238  DCTN6; dynactin subunit 6 [KO:K10428]
100020379  UQCRC1; cytochrome b-c1 complex subunit 1, mitochondrial [KO:K00414]
100020481  [KO:K02267]
100020514  PSMD7; proteasome 26S subunit, non-ATPase 7 [KO:K03038]
100020665  ATG14; autophagy related 14 [KO:K17889]
100020666  ADRM1; adhesion regulating molecule 1 [KO:K06691]
100020711  BBC3; BCL2 binding component 3 [KO:K10132]
100020933  NDUFS7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2]
100021036  TUBB2B; tubulin beta 2B class IIb [KO:K07375]
100021064  TUBB2A; tubulin beta-2A chain [KO:K07375]
100021079  AP2S1; adaptor related protein complex 2 sigma 1 subunit [KO:K11827]
100021105  ACTR10; actin-related protein 10 homolog [KO:K16576]
100021131  PSMA3; proteasome subunit alpha 3 [KO:K02727] [EC:3.4.25.1]
100021402  ATP5E; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135]
100021718  PSMD9; proteasome 26S subunit, non-ATPase 9 [KO:K06693]
100022173  ATP5D; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134]
100022184  [KO:K02267]
100022210  TUBA8; tubulin alpha 8 [KO:K07374]
100022547  CREB3L3; cAMP responsive element binding protein 3 like 3 [KO:K09048]
100022562  TUBB; tubulin beta class I [KO:K07375]
100022645  ATP5J; ATP synthase, H+ transporting, mitochondrial Fo complex subunit F6 [KO:K02131]
100022680  GPX2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
100022756  HDAC2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
100022923  ACTR1A; ARP1 actin-related protein 1 homolog A, centractin alpha [KO:K16575]
100023062  [KO:K02267]
100023144  RB1CC1; RB1 inducible coiled-coil 1 [KO:K17589]
100023256  KCNJ10; potassium voltage-gated channel subfamily J member 10 [KO:K05003]
100023278  ATP5H; ATP synthase, H+ transporting, mitochondrial Fo complex subunit D [KO:K02138]
100023318  KIF5B; kinesin family member 5B [KO:K10396]
100023403  POLR2K; RNA polymerase II subunit K [KO:K03009]
100023485  GRIN1; glutamate ionotropic receptor NMDA type subunit 1 [KO:K05208]
100023645  GRIA2; glutamate ionotropic receptor AMPA type subunit 2 [KO:K05198]
100023836  PIK3R4; phosphoinositide-3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
100023896  CACNA1B; calcium voltage-gated channel subunit alpha1 B [KO:K04849]
100023986  NDUFB2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
100023995  [KO:K00416]
100024092  PSMB2; proteasome subunit beta 2 [KO:K02734] [EC:3.4.25.1]
100024148  DNAH10; dynein axonemal heavy chain 10 [KO:K10408]
100024259  DCTN4; dynactin subunit 4 [KO:K10426]
100024279  NDUFB6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
100024430  DNAL1; dynein axonemal light chain 1 [KO:K10411]
100024500  WIPI1; WD repeat domain, phosphoinositide interacting 1 [KO:K17908]
100024672  [KO:K07375]
100024757  ACTR1B; ARP1 actin-related protein 1 homolog B, centractin beta [KO:K16575]
100024776  PSMD12; proteasome 26S subunit, non-ATPase 12 [KO:K03035]
100024981  NDUFS5; NADH:ubiquinone oxidoreductase subunit S5 [KO:K03938]
100025091  PPARG; peroxisome proliferator activated receptor gamma [KO:K08530]
100025322  NDUFA11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
100025610  NDUFA4; NDUFA4, mitochondrial complex associated [KO:K03948]
100025645  TUBA3E; tubulin alpha 3e [KO:K07374]
100025773  TBPL2; TATA-box binding protein like 2 [KO:K03120]
100025820  PSMA4; proteasome subunit alpha 4 [KO:K02728] [EC:3.4.25.1]
100025951  TUBB4A; tubulin beta 4A class IVa [KO:K07375]
100025973  PSMD2; proteasome 26S subunit, non-ATPase 2 [KO:K03028]
100026076  ATP5O; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137]
100026117  [KO:K02266]
100026210  NDUFB9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
100026270  PSMC5; proteasome 26S subunit, ATPase 5 [KO:K03066]
100026327  PSMA7; proteasome subunit alpha 7 [KO:K02731] [EC:3.4.25.1]
100026394  NDUFA2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
100026417  TAF4; TATA-box binding protein associated factor 4 [KO:K03129]
100026418  ERN1; endoplasmic reticulum to nucleus signaling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
100026423  NDUFB7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
100026476  DNAH3; dynein axonemal heavy chain 3 [KO:K10408]
100026672  UQCRC2; cytochrome b-c1 complex subunit 2, mitochondrial [KO:K00415]
100026752  MAP2K7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
100026811  [KO:K08738]
100027039  TUBB1; tubulin beta 1 class VI [KO:K07375]
100027109  GRIA4; glutamate ionotropic receptor AMPA type subunit 4 [KO:K05200]
100027336  NDUFC2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
100027338  CASP9; caspase 9 [KO:K04399] [EC:3.4.22.62]
100027439  CLTB; clathrin light chain B [KO:K04645]
100027445  [KO:K03129]
100027632  TUBB3; tubulin beta 3 class III [KO:K07375]
100027682  [KO:K02137]
100028089  MAPK9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
100028102  EP300; E1A binding protein p300 [KO:K04498] [EC:2.3.1.48]
100028224  DNAI2; dynein axonemal intermediate chain 2 [KO:K11143]
100028481  ULK1; unc-51 like autophagy activating kinase 1 [KO:K21357] [EC:2.7.11.1]
100028584  NDUFA3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
100028736  TBP; TATA-box binding protein [KO:K03120]
100028844  DCTN3; dynactin subunit 3 [KO:K10425]
100028940  DNAI1; dynein axonemal intermediate chain 1 [KO:K10409]
100028959  NDUFS2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2]
100029192  PSMA1; proteasome subunit alpha 1 [KO:K02725] [EC:3.4.25.1]
100029287  CLTCL1; clathrin heavy chain like 1 [KO:K04646]
100029378  GPX3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
100029525  DNAH7; dynein axonemal heavy chain 7 [KO:K10408]
100029560  STX1A; syntaxin 1A [KO:K04560]
100029673  GRIA1; glutamate ionotropic receptor AMPA type subunit 1 [KO:K05197]
100029746  DNAH8; dynein axonemal heavy chain 8 [KO:K10408]
100029750  KLC2; kinesin light chain 2 [KO:K10407]
100030019  GRIA3; glutamate ionotropic receptor AMPA type subunit 3 [KO:K05199]
100030041  PSMA5; proteasome subunit alpha 5 [KO:K02729] [EC:3.4.25.1]
100030045  [KO:K02264]
100030139  [KO:K00417]
100030164  VDAC3; voltage dependent anion channel 3 [KO:K15041]
100030198  AP2A1; adaptor related protein complex 2 alpha 1 subunit [KO:K11824]
100030336  [KO:K02265]
100030412  BAX; BCL2 associated X, apoptosis regulator [KO:K02159]
100030419  SIN3A; SIN3 transcription regulator family member A [KO:K11644]
100030431  PSMB5; proteasome subunit beta 5 [KO:K02737] [EC:3.4.25.1]
100030487  [KO:K02137]
100030489  [KO:K02268]
100030590  DNAH6; dynein axonemal heavy chain 6 [KO:K10408]
100030680  [KO:K05863]
100030755  PSMD13; proteasome 26S subunit, non-ATPase 13 [KO:K03039]
100030784  [KO:K03939]
100030790  [KO:K00419]
100030966  DNAH1; dynein axonemal heavy chain 1 [KO:K10408]
100031028  DNAH17; dynein axonemal heavy chain 17 [KO:K10408]
100031263  PLCB3; phospholipase C beta 3 [KO:K05858] [EC:3.1.4.11]
100031385  TBPL1; TATA-box binding protein like 1 [KO:K03120]
100031475  DNAH9; dynein axonemal heavy chain 9 [KO:K10408]
100031527  MAP3K5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
100031559  NDUFS4; NADH:ubiquinone oxidoreductase subunit S4 [KO:K03937]
100031660  PLCB1; phospholipase C beta 1 [KO:K05858] [EC:3.1.4.11]
100031703  DNALI1; dynein axonemal light intermediate chain 1 [KO:K10410]
100031869  PLCB2; phospholipase C beta 2 [KO:K05858] [EC:3.1.4.11]
100031958  [KO:K05863]
100032261  SDHD; succinate dehydrogenase complex subunit D [KO:K00237]
100032291  [KO:K02270]
100032301  HDAC1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
100032426  SOD2; superoxide dismutase 2 [KO:K04564] [EC:1.15.1.1]
100032634  CREB5; cAMP responsive element binding protein 5 [KO:K09047]
100032711  PSMB1; proteasome subunit beta 1 [KO:K02732] [EC:3.4.25.1]
100032780  SDHC; succinate dehydrogenase complex subunit C [KO:K00236]
100032853  TGM2; transglutaminase 2 [KO:K05625] [EC:2.3.2.13]
100033122  ATP5F1; ATP synthase, H+ transporting, mitochondrial Fo complex subunit B1 [KO:K02127]
100033281  PLCB4; phospholipase C beta 4 [KO:K05858] [EC:3.1.4.11]
100192384  PSMD4; proteasome 26S subunit, non-ATPase 4 [KO:K03029]
100616881  DNAH12; dynein axonemal heavy chain 12 [KO:K10408]
100617599  CASP8; caspase-8 [KO:K04398] [EC:3.4.22.61]
100618050  NDUFB11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
100618273  [KO:K02270]
100618522  NDUFS8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2]
100618895  MAPK10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
100619178  DNAH14; dynein axonemal heavy chain 14 [KO:K10408]
100619445  NDUFA10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
100619675  MTOR; mechanistic target of rapamycin [KO:K07203] [EC:2.7.11.1]
100619791  GPX1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
103096959  [KO:K02132]
103098724  TFAM; transcription factor A, mitochondrial [KO:K11830]
103099076  [KO:K02267]
103100668  NDUFA1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
103103819  NDUFV3; NADH:ubiquinone oxidoreductase subunit V3 [KO:K03944]
103104366  CREB3; cAMP responsive element binding protein 3 [KO:K09048]
103105219  [KO:K02273]
103106696  [KO:K02263]
3074656  ND4; NADH dehydrogenase subunit 4 [KO:K03881] [EC:7.1.1.2]
3074657  ND4L; NADH dehydrogenase subunit 4L [KO:K03882] [EC:7.1.1.2]
3074658  ND3; NADH dehydrogenase subunit 3 [KO:K03880] [EC:7.1.1.2]
3074659  CYTB; cytochrome b [KO:K00412]
3074660  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
3074661  ND1; NADH dehydrogenase subunit 1 [KO:K03878] [EC:7.1.1.2]
3074662  COX3; cytochrome c oxidase subunit III [KO:K02262]
3074663  COX2; cytochrome c oxidase subunit II [KO:K02261]
3074664  ND6; NADH dehydrogenase subunit 6 [KO:K03884] [EC:7.1.1.2]
3074665  ND5; NADH dehydrogenase subunit 5 [KO:K03883] [EC:7.1.1.2]
3074666  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
3074667  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
3074668  ND2; NADH dehydrogenase subunit 2 [KO:K03879] [EC:7.1.1.2]
497248  TP53; tumor protein p53 [KO:K04451]
554233  BDNF; brain derived neurotrophic factor [KO:K04355]
654273  CASP3; caspase 3 [KO:K02187] [EC:3.4.22.56]
654275  [KO:K02187] [EC:3.4.22.56]
751078  SDHB; succinate dehydrogenase complex iron sulfur subunit B [KO:K00235] [EC:1.3.5.1]
751085  SDHA; succinate dehydrogenase complex flavoprotein subunit A [KO:K00234] [EC:1.3.5.1]
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
mdo00190  Oxidative phosphorylation
mdo03020  RNA polymerase
mdo03022  Basal transcription factors
mdo03050  Proteasome
mdo04020  Calcium signaling pathway
mdo04115  p53 signaling pathway
mdo04140  Autophagy - animal
mdo04141  Protein processing in endoplasmic reticulum
mdo04144  Endocytosis
mdo04210  Apoptosis
mdo04724  Glutamatergic synapse
KO pathway
ko05016   

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