KEGG   PATHWAY: mmu05016Help
Entry
mmu05016                    Pathway                                

Name
Huntington disease - Mus musculus (mouse)
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
BRITE hierarchy
Pathway map
mmu05016  Huntington disease
mmu05016

Ortholog table
Organism
Mus musculus (mouse) [GN:mmu]
Gene
12757  Clta; clathrin, light polypeptide (Lca) [KO:K04644]
74325  Cltb; clathrin, light polypeptide (Lcb) [KO:K04645]
67300  Cltc; clathrin, heavy polypeptide (Hc) [KO:K04646]
215114  Hip1; huntingtin interacting protein 1 [KO:K04559]
11772  Ap2a2; adaptor-related protein complex 2, alpha 2 subunit [KO:K11824]
11771  Ap2a1; adaptor-related protein complex 2, alpha 1 subunit [KO:K11824]
71770  Ap2b1; adaptor-related protein complex 2, beta 1 subunit [KO:K11825]
11773  Ap2m1; adaptor-related protein complex 2, mu 1 subunit [KO:K11826]
232910  Ap2s1; adaptor-related protein complex 2, sigma 1 subunit [KO:K11827]
15194  Htt; huntingtin [KO:K04533]
13191  Dctn1; dynactin 1 [KO:K04648]
69654  Dctn2; dynactin 2 [KO:K10424]
53598  Dctn3; dynactin 3 [KO:K10425]
67665  Dctn4; dynactin 4 [KO:K10426]
59288  Dctn5; dynactin 5 [KO:K10427]
22428  Dctn6; dynactin 6 [KO:K10428]
54130  Actr1a; ARP1 actin-related protein 1A, centractin alpha [KO:K16575]
226977  Actr1b; ARP1 actin-related protein 1B, centractin beta [KO:K16575]
56444  Actr10; ARP10 actin-related protein 10 [KO:K16576]
15114  Hap1; huntingtin-associated protein 1 [KO:K04647]
110084  Dnah1; dynein, axonemal, heavy chain 1 [KO:K10408]
381917  Dnah3; dynein, axonemal, heavy chain 3 [KO:K10408]
327954  Dnah2; dynein, axonemal, heavy chain 2 [KO:K10408]
227058  Dnah7b; dynein, axonemal, heavy chain 7B [KO:K10408]
110082  Dnah5; dynein, axonemal, heavy chain 5 [KO:K10408]
330355  Dnah6; dynein, axonemal, heavy chain 6 [KO:K10408]
237806  Dnah9; dynein, axonemal, heavy chain 9 [KO:K10408]
13411  Dnah11; dynein, axonemal, heavy chain 11 [KO:K10408]
110083  Dnah12; dynein, axonemal, heavy chain 12 [KO:K10408]
13417  Dnah8; dynein, axonemal, heavy chain 8 [KO:K10408]
627872  Dnah7a; dynein, axonemal, heavy chain 7A [KO:K10408]
56087  Dnah10; dynein, axonemal, heavy chain 10 [KO:K10408]
69926  Dnah17; dynein, axonemal, heavy chain 17 [KO:K10408]
240960  Dnah14; dynein, axonemal, heavy chain 14 [KO:K10408]
100101919  Dnah7c; dynein, axonemal, heavy chain 7C [KO:K10408]
75563  Dnali1; dynein, axonemal, light intermediate polypeptide 1 [KO:K10410]
68922  Dnaic1; dynein, axonemal, intermediate chain 1 [KO:K10409]
432611  Dnaic2; dynein, axonemal, intermediate chain 2 [KO:K11143]
105000  Dnal1; dynein, axonemal, light chain 1 [KO:K10411]
54152  Dnal4; dynein, axonemal, light chain 4 [KO:K10412]
22145  Tuba4a; tubulin, alpha 4A [KO:K07374]
22144  Tuba3a; tubulin, alpha 3A [KO:K07374]
22142  Tuba1a; tubulin, alpha 1A [KO:K07374]
22146  Tuba1c; tubulin, alpha 1C [KO:K07374]
22147  Tuba3b; tubulin, alpha 3B [KO:K07374]
22143  Tuba1b; tubulin, alpha 1B [KO:K07374]
53857  Tuba8; tubulin, alpha 8 [KO:K07374]
238463  Tubal3; tubulin, alpha-like 3 [KO:K07374]
227613  Tubb4b; tubulin, beta 4B class IVB [KO:K07375]
22151  Tubb2a; tubulin, beta 2A class IIA [KO:K07375]
22152  Tubb3; tubulin, beta 3 class III [KO:K07375]
22153  Tubb4a; tubulin, beta 4A class IVA [KO:K07375]
67951  Tubb6; tubulin, beta 6 class V [KO:K07375]
22154  Tubb5; tubulin, beta 5 class I [KO:K07375]
545486  Tubb1; tubulin, beta 1 class VI [KO:K07375]
73710  Tubb2b; tubulin, beta 2B class IIB [KO:K07375]
26419  Mapk8; mitogen-activated protein kinase 8 [KO:K04440] [EC:2.7.11.24]
26420  Mapk9; mitogen-activated protein kinase 9 [KO:K04440] [EC:2.7.11.24]
26414  Mapk10; mitogen-activated protein kinase 10 [KO:K04440] [EC:2.7.11.24]
16572  Kif5a; kinesin family member 5A [KO:K10396]
16573  Kif5b; kinesin family member 5B [KO:K10396]
16574  Kif5c; kinesin family member 5C [KO:K10396]
16593  Klc1; kinesin light chain 1 [KO:K10407]
16594  Klc2; kinesin light chain 2 [KO:K10407]
232943  Klc3; kinesin light chain 3 [KO:K10407]
74764  Klc4; kinesin light chain 4 [KO:K10407]
73916  Ift57; intraflagellar transport 57 [KO:K04638]
12370  Casp8; caspase 8 [KO:K04398] [EC:3.4.22.61]
12367  Casp3; caspase 3 [KO:K02187] [EC:3.4.22.56]
108071  Grm5; glutamate receptor, metabotropic 5 [KO:K04604]
14682  Gnaq; guanine nucleotide binding protein, alpha q polypeptide [KO:K04634]
18795  Plcb1; phospholipase C, beta 1 [KO:K05858] [EC:3.1.4.11]
18797  Plcb3; phospholipase C, beta 3 [KO:K05858] [EC:3.1.4.11]
18798  Plcb4; phospholipase C, beta 4 [KO:K05858] [EC:3.1.4.11]
18796  Plcb2; phospholipase C, beta 2 [KO:K05858] [EC:3.1.4.11]
20907  Stx1a; syntaxin 1A (brain) [KO:K04560]
12287  Cacna1b; calcium channel, voltage-dependent, N type, alpha 1B subunit [KO:K04849]
14810  Grin1; glutamate receptor, ionotropic, NMDA1 (zeta 1) [KO:K05208]
14812  Grin2b; glutamate receptor, ionotropic, NMDA2B (epsilon 2) [KO:K05210]
16438  Itpr1; inositol 1,4,5-trisphosphate receptor 1 [KO:K04958]
13385  Dlg4; discs large MAGUK scaffold protein 4 [KO:K11828]
14799  Gria1; glutamate receptor, ionotropic, AMPA1 (alpha 1) [KO:K05197]
14800  Gria2; glutamate receptor, ionotropic, AMPA2 (alpha 2) [KO:K05198]
53623  Gria3; glutamate receptor, ionotropic, AMPA3 (alpha 3) [KO:K05199]
14802  Gria4; glutamate receptor, ionotropic, AMPA4 (alpha 4) [KO:K05200]
21817  Tgm2; transglutaminase 2, C polypeptide [KO:K05625] [EC:2.3.2.13]
20512  Slc1a3; solute carrier family 1 (glial high affinity glutamate transporter), member 3 [KO:K05614]
20511  Slc1a2; solute carrier family 1 (glial high affinity glutamate transporter), member 2 [KO:K05613]
16513  Kcnj10; potassium inwardly-rectifying channel, subfamily J, member 10 [KO:K05003]
19712  Rest; RE1-silencing transcription factor [KO:K09222]
20466  Sin3a; transcriptional regulator, SIN3A (yeast) [KO:K11644]
217864  Rcor1; REST corepressor 1 [KO:K11829]
15182  Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
433759  Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
20020  Polr2a; polymerase (RNA) II (DNA directed) polypeptide A [KO:K03006] [EC:2.7.7.6]
231329  Polr2b; polymerase (RNA) II (DNA directed) polypeptide B [KO:K03010] [EC:2.7.7.6]
20021  Polr2c; polymerase (RNA) II (DNA directed) polypeptide C [KO:K03011]
69241  Polr2d; polymerase (RNA) II (DNA directed) polypeptide D [KO:K03012]
66420  Polr2e; polymerase (RNA) II (DNA directed) polypeptide E [KO:K03013]
69833  Polr2f; polymerase (RNA) II (DNA directed) polypeptide F [KO:K03014]
67710  Polr2g; polymerase (RNA) II (DNA directed) polypeptide G [KO:K03015]
245841  Polr2h; polymerase (RNA) II (DNA directed) polypeptide H [KO:K03016]
69920  Polr2i; polymerase (RNA) II (DNA directed) polypeptide I [KO:K03017]
66491  Polr2l; polymerase (RNA) II (DNA directed) polypeptide L [KO:K03007]
20022  Polr2j; polymerase (RNA) II (DNA directed) polypeptide J [KO:K03008]
100862456  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
102641086  DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
12064  Bdnf; brain derived neurotrophic factor [KO:K04355]
12914  Crebbp; CREB binding protein [KO:K04498] [EC:2.3.1.48]
328572  Ep300; E1A binding protein p300 [KO:K04498] [EC:2.3.1.48]
72504  Taf4b; TATA-box binding protein associated factor 4b [KO:K03129]
228980  Taf4; TATA-box binding protein associated factor 4 [KO:K03129]
21374  Tbp; TATA box binding protein [KO:K03120]
237336  Tbpl1; TATA box binding protein-like 1 [KO:K03120]
227606  Tbpl2; TATA box binding protein like 2 [KO:K03120]
20683  Sp1; trans-acting transcription factor 1 [KO:K04684]
12912  Creb1; cAMP responsive element binding protein 1 [KO:K05870]
12913  Creb3; cAMP responsive element binding protein 3 [KO:K09048]
26427  Creb3l1; cAMP responsive element binding protein 3-like 1 [KO:K09048]
208647  Creb3l2; cAMP responsive element binding protein 3-like 2 [KO:K09048]
208677  Creb3l3; cAMP responsive element binding protein 3-like 3 [KO:K09048]
78284  Creb3l4; cAMP responsive element binding protein 3-like 4 [KO:K09048]
231991  Creb5; cAMP responsive element binding protein 5 [KO:K09047]
19017  Ppargc1a; peroxisome proliferative activated receptor, gamma, coactivator 1 alpha [KO:K07202]
19016  Pparg; peroxisome proliferator activated receptor gamma [KO:K08530]
21780  Tfam; transcription factor A, mitochondrial [KO:K11830]
18181  Nrf1; nuclear respiratory factor 1 [KO:K11831]
20655  Sod1; superoxide dismutase 1, soluble [KO:K04565] [EC:1.15.1.1]
20656  Sod2; superoxide dismutase 2, mitochondrial [KO:K04564] [EC:1.15.1.1]
14775  Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]
14776  Gpx2; glutathione peroxidase 2 [KO:K00432] [EC:1.11.1.9]
14778  Gpx3; glutathione peroxidase 3 [KO:K00432] [EC:1.11.1.9]
14780  Gpx5; glutathione peroxidase 5 [KO:K00432] [EC:1.11.1.9]
67305  Gpx7; glutathione peroxidase 7 [KO:K00432] [EC:1.11.1.9]
75512  Gpx6; glutathione peroxidase 6 [KO:K00432] [EC:1.11.1.9]
69590  Gpx8; glutathione peroxidase 8 (putative) [KO:K00432] [EC:1.11.1.9]
22227  Ucp1; uncoupling protein 1 (mitochondrial, proton carrier) [KO:K08769]
22059  Trp53; transformation related protein 53 [KO:K04451]
12028  Bax; BCL2-associated X protein [KO:K02159]
170770  Bbc3; BCL2 binding component 3 [KO:K10132]
17995  Ndufv1; NADH:ubiquinone oxidoreductase core subunit V1 [KO:K03942] [EC:7.1.1.2 1.6.99.3]
72900  Ndufv2; NADH:ubiquinone oxidoreductase core subunit V2 [KO:K03943] [EC:7.1.1.2 1.6.99.3]
78330  Ndufv3; NADH:ubiquinone oxidoreductase core subunit V3 [KO:K03944]
54405  Ndufa1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945]
17991  Ndufa2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946]
66091  Ndufa3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947]
17992  Ndufa4; Ndufa4, mitochondrial complex associated [KO:K03948]
407790  Ndufa4l2; Ndufa4, mitochondrial complex associated like 2 [KO:K03948]
68202  Ndufa5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949]
67130  Ndufa6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950]
66416  Ndufa7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951]
68375  Ndufa8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952]
66108  Ndufa9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953]
67273  Ndufa10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954]
70316  Ndufab1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955]
69875  Ndufa11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956]
66414  Ndufa12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352]
67184  Ndufa13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353]
68198  Ndufb2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958]
66495  Ndufb3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959]
68194  Ndufb4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960]
100042503  Ndufb4b; NADH:ubiquinone oxidoreductase subunit B4B [KO:K03960]
100041273  Ndufb4c; NADH:ubiquinone oxidoreductase subunit B4C [KO:K03960]
66046  Ndufb5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961]
230075  Ndufb6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962]
66916  Ndufb7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963]
67264  Ndufb8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964]
66218  Ndufb9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965]
68342  Ndufb10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966]
104130  Ndufb11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351]
227197  Ndufs1; NADH:ubiquinone oxidoreductase core subunit S1 [KO:K03934] [EC:7.1.1.2 1.6.99.3]
226646  Ndufs2; NADH:ubiquinone oxidoreductase core subunit S2 [KO:K03935] [EC:7.1.1.2 1.6.99.3]
68349  Ndufs3; NADH:ubiquinone oxidoreductase core subunit S3 [KO:K03936] [EC:7.1.1.2 1.6.99.3]
17993  Ndufs4; NADH:ubiquinone oxidoreductase core subunit S4 [KO:K03937]
595136  Ndufs5; NADH:ubiquinone oxidoreductase core subunit S5 [KO:K03938]
407785  Ndufs6; NADH:ubiquinone oxidoreductase core subunit S6 [KO:K03939]
75406  Ndufs7; NADH:ubiquinone oxidoreductase core subunit S7 [KO:K03940] [EC:7.1.1.2 1.6.99.3]
225887  Ndufs8; NADH:ubiquinone oxidoreductase core subunit S8 [KO:K03941] [EC:7.1.1.2 1.6.99.3]
66377  Ndufc1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967]
68197  Ndufc2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968]
66945  Sdha; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234] [EC:1.3.5.1]
67680  Sdhb; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235] [EC:1.3.5.1]
66052  Sdhc; succinate dehydrogenase complex, subunit C, integral membrane protein [KO:K00236]
66925  Sdhd; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237]
66694  Uqcrfs1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:7.1.1.8]
17711  CYTB; cytochrome b [KO:K00412]
66445  Cyc1; cytochrome c-1 [KO:K00413]
22273  Uqcrc1; ubiquinol-cytochrome c reductase core protein 1 [KO:K00414]
67003  Uqcrc2; ubiquinol cytochrome c reductase core protein 2 [KO:K00415]
66576  Uqcrh; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
67530  Uqcrb; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
22272  Uqcrq; ubiquinol-cytochrome c reductase, complex III subunit VII [KO:K00418]
66152  Uqcr10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
66594  Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
17710  COX3; cytochrome c oxidase subunit III [KO:K02262]
17708  COX1; cytochrome c oxidase subunit I [KO:K02256] [EC:7.1.1.9]
17709  COX2; cytochrome c oxidase subunit II [KO:K02261]
12857  Cox4i1; cytochrome c oxidase subunit 4I1 [KO:K02263]
84682  Cox4i2; cytochrome c oxidase subunit 4I2 [KO:K02263]
12858  Cox5a; cytochrome c oxidase subunit 5A [KO:K02264]
12859  Cox5b; cytochrome c oxidase subunit 5B [KO:K02265]
12861  Cox6a1; cytochrome c oxidase subunit 6A1 [KO:K02266]
12862  Cox6a2; cytochrome c oxidase subunit 6A2 [KO:K02266]
110323  Cox6b1; cytochrome c oxidase, subunit 6B1 [KO:K02267]
333182  Cox6b2; cytochrome c oxidase subunit 6B2 [KO:K02267]
12864  Cox6c; cytochrome c oxidase subunit 6C [KO:K02268]
12866  Cox7a2; cytochrome c oxidase subunit 7A2 [KO:K02270]
12865  Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270]
20463  Cox7a2l; cytochrome c oxidase subunit 7A2 like [KO:K02270]
66142  Cox7b; cytochrome c oxidase subunit 7B [KO:K02271]
78174  Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271]
12867  Cox7c; cytochrome c oxidase subunit 7C [KO:K02272]
12868  Cox8a; cytochrome c oxidase subunit 8A [KO:K02273]
12869  Cox8b; cytochrome c oxidase subunit 8B [KO:K02273]
75483  Cox8c; cytochrome c oxidase subunit 8C [KO:K02273]
11946  Atp5a1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1 [KO:K02132]
11947  Atp5b; ATP synthase, H+ transporting mitochondrial F1 complex, beta subunit [KO:K02133] [EC:7.1.2.2]
11949  Atp5c1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136]
66043  Atp5d; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134]
67126  Atp5e; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135]
17705  ATP6; ATP synthase F0 subunit 6 [KO:K02126]
11950  Atp5pb; ATP synthase peripheral stalk-membrane subunit b [KO:K02127]
11951  Atp5g1; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C1 (subunit 9) [KO:K02128]
67942  Atp5g2; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C2 (subunit 9) [KO:K02128]
228033  Atp5g3; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C3 (subunit 9) [KO:K02128]
71679  Atp5h; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit D [KO:K02138]
28080  Atp5o; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137]
11957  Atp5j; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit F [KO:K02131]
17706  ATP8; ATP synthase F0 subunit 8 [KO:K02125]
22333  Vdac1; voltage-dependent anion channel 1 [KO:K05862]
22334  Vdac2; voltage-dependent anion channel 2 [KO:K15040]
22335  Vdac3; voltage-dependent anion channel 3 [KO:K15041]
11739  Slc25a4; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 4 [KO:K05863]
11740  Slc25a5; solute carrier family 25 (mitochondrial carrier, adenine nucleotide translocator), member 5 [KO:K05863]
73333  Slc25a31; solute carrier family 25 (mitochondrial carrier; adenine nucleotide translocator), member 31 [KO:K05863]
105675  Ppif; peptidylprolyl isomerase F (cyclophilin F) [KO:K09565] [EC:5.2.1.8]
13063  Cycs; cytochrome c, somatic [KO:K08738]
13067  Cyct; cytochrome c, testis [KO:K08738]
11783  Apaf1; apoptotic peptidase activating factor 1 [KO:K02084]
12371  Casp9; caspase 9 [KO:K04399] [EC:3.4.22.62]
269881  Map3k10; mitogen-activated protein kinase kinase kinase 10 [KO:K04418] [EC:2.7.11.25]
78943  Ern1; endoplasmic reticulum (ER) to nucleus signalling 1 [KO:K08852] [EC:2.7.11.1 3.1.26.-]
22030  Traf2; TNF receptor-associated factor 2 [KO:K03173] [EC:2.3.2.27]
26408  Map3k5; mitogen-activated protein kinase kinase kinase 5 [KO:K04426] [EC:2.7.11.25]
26400  Map2k7; mitogen-activated protein kinase kinase 7 [KO:K04431] [EC:2.7.12.2]
56717  Mtor; mechanistic target of rapamycin kinase [KO:K07203] [EC:2.7.11.1]
22241  Ulk1; unc-51 like kinase 1 [KO:K21357] [EC:2.7.11.1]
29869  Ulk2; unc-51 like kinase 2 [KO:K08269] [EC:2.7.11.1]
68118  Atg101; autophagy related 101 [KO:K19730]
51897  Atg13; autophagy related 13 [KO:K08331]
12421  Rb1cc1; RB1-inducible coiled-coil 1 [KO:K17589]
228361  Ambra1; autophagy/beclin 1 regulator 1 [KO:K17985]
56208  Becn1; beclin 1, autophagy related [KO:K08334]
75669  Pik3r4; phosphoinositide-3-kinase regulatory subunit 4 [KO:K08333] [EC:2.7.11.1]
100504663  Atg14; autophagy related 14 [KO:K17889]
641340  Nrbf2; nuclear receptor binding factor 2 [KO:K21246]
225326  Pik3c3; phosphatidylinositol 3-kinase catalytic subunit type 3 [KO:K00914] [EC:2.7.1.137]
329015  Atg2a; autophagy related 2A [KO:K17906]
76559  Atg2b; autophagy related 2B [KO:K17906]
52639  Wipi1; WD repeat domain, phosphoinositide interacting 1 [KO:K17908]
74781  Wipi2; WD repeat domain, phosphoinositide interacting 2 [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
  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)
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Borrell-Pages M, Zala D, Humbert S, Saudou F
  Title
Huntington's disease: from huntingtin function and dysfunction to therapeutic strategies.
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Cell Mol Life Sci 63:2642-60 (2006)
DOI:10.1007/s00018-006-6242-0
Reference
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Bossy-Wetzel E, Petrilli A, Knott AB
  Title
Mutant huntingtin and mitochondrial dysfunction.
  Journal
Trends Neurosci 31:609-16 (2008)
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Reference
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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
  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
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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
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Ross CA.
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Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders.
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Neuron 35:819-22 (2002)
DOI:10.1016/S0896-6273(02)00872-3
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Ross CA
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Huntington's disease: new paths to pathogenesis.
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Cell 118:4-7 (2004)
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Coffey ET
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Nuclear and cytosolic JNK signalling in neurons.
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Nat Rev Neurosci 15:285-99 (2014)
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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.
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Nat Neurosci 12:864-71 (2009)
DOI:10.1038/nn.2346
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Kumar P, Kumar D, Jha SK, Jha NK, Ambasta RK
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Ion Channels in Neurological Disorders.
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Adv Protein Chem Struct Biol 103:97-136 (2016)
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Proft J, Weiss N
  Title
Rectifying rectifier channels in Huntington disease.
  Journal
Commun Integr Biol 7:e29410 (2014)
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Tang TS, Slow E, Lupu V, Stavrovskaya IG, Sugimori M, Llinas R, Kristal BS, Hayden MR, Bezprozvanny I
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Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
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Proc Natl Acad Sci U S A 102:2602-7 (2005)
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Mackay JP, Nassrallah WB, Raymond LA
  Title
Cause or compensation?-Altered neuronal Ca(2+) handling in Huntington's disease.
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CNS Neurosci Ther 24:301-310 (2018)
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Mattson MP.
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Accomplices to neuronal death.
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Nature 415:377-9 (2002)
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McGill JK, Beal MF
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PGC-1alpha, a new therapeutic target in Huntington's disease?
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Cell 127:465-8 (2006)
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Intihar TA, Martinez EA, Gomez-Pastor R
  Title
Mitochondrial Dysfunction in Huntington's Disease; Interplay Between HSF1, p53 and PGC-1alpha Transcription Factors.
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Front Cell Neurosci 13:103 (2019)
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La Spada AR, Morrison RS
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The power of the dark side: Huntington's disease protein and p53 form a deadly alliance.
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Neuron 47:1-3 (2005)
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Lesort M, Chun W, Tucholski J, Johnson GV
  Title
Does tissue transglutaminase play a role in Huntington's disease?
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Neurochem Int 40:37-52 (2002)
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Bae BI, Xu H, Igarashi S, Fujimuro M, Agrawal N, Taya Y, Hayward SD, Moran TH, Montell C, Ross CA, Snyder SH, Sawa A
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p53 mediates cellular dysfunction and behavioral abnormalities in Huntington's disease.
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Neuron 47:29-41 (2005)
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Li SH, Cheng AL, Zhou H, Lam S, Rao M, Li H, Li XJ
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Interaction of Huntington disease protein with transcriptional activator Sp1.
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Mol Cell Biol 22:1277-87 (2002)
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Vidal RL, Matus S, Bargsted L, Hetz C
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Targeting autophagy in neurodegenerative diseases.
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Trends Pharmacol Sci 35:583-91 (2014)
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Reference
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Shibata M, Lu T, Furuya T, Degterev A, Mizushima N, Yoshimori T, MacDonald M, Yankner B, Yuan J
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Regulation of intracellular accumulation of mutant Huntingtin by Beclin 1.
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J Biol Chem 281:14474-85 (2006)
DOI:10.1074/jbc.M600364200
Related
pathway
mmu00190  Oxidative phosphorylation
mmu03020  RNA polymerase
mmu03022  Basal transcription factors
mmu03050  Proteasome
mmu04020  Calcium signaling pathway
mmu04115  p53 signaling pathway
mmu04140  Autophagy - animal
mmu04141  Protein processing in endoplasmic reticulum
mmu04144  Endocytosis
mmu04210  Apoptosis
mmu04724  Glutamatergic synapse
KO pathway
ko05016   

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