KEGG PATHWAY: mmu05016

PATHWAY: mmu05016

Entry

mmu05016 Pathway

Name

Huntington's 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 IT15gene, which results in a long stretch of polyglutamine close to the amino-terminus of the HD protein huntingtin (Htt). Mutant Htt (mHtt) has effects both in the cytoplasm and in the nucleus. In the cytoplasm, full-length mHtt can interfere with BDNF vesicular transport on microtubules. This mutant protein also may lead to abnormal endocytosis and secretion in neurons, because normal Htt form a complex with the proteins Hip1, clathrin and AP2 that are involved in endocytosis. In addition, mHtt affects Ca2+ signaling by sensitizing InsP3R1 to activation by InsP3, stimulating NMDAR activity, and destabilizing mitochondrial Ca2+ handling. The mHtt translocates to the nucleus, where it forms intranuclear inclusions. Nuclear toxicity is believed to be caused by interference with gene transcription, leading to loss of transcription of neuroprotective molecules such as BDNF. While mHtt binds to p53 and upregulates levels of nuclear p53 as well as p53 transcriptional activity. Augmented p53 mediates mitochondrial dysfunction.

Class

Human Diseases; Neurodegenerative diseases

Pathway map

mmu05016

Huntington's disease

Module

mmu_M00180

RNA polymerase II, eukaryotes [PATH:mmu05016]

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]
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]
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 homolog 4 (Drosophila) [KO:K11828]
21817	Tgm2; transglutaminase 2, C polypeptide [KO:K05625] [EC:2.3.2.13]
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; K03009 DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
12064	Bdnf; brain derived neurotrophic factor [KO:K04355]
13191	Dctn1; dynactin 1 [KO:K04648]
69654	Dctn2; dynactin 2 [KO:K10424]
67665	Dctn4; dynactin 4 [KO:K10426]
15114	Hap1; huntingtin-associated protein 1 [KO:K04647]
110084	Dnahc1; dynein, axonemal, heavy chain 1 [KO:K10408]
381917	Dnahc3; dynein, axonemal, heavy chain 3 [KO:K10408]
327954	Dnahc2; dynein, axonemal, heavy chain 2 [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	Dnalc1; dynein, axonemal, light chain 1 [KO:K10411]
54152	Dnalc4; dynein, axonemal, light chain 4 [KO:K10412]
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; TAF4B RNA polymerase II, TATA box binding protein (TBP)-associated factor [KO:K03129]
228980	Taf4a; TAF4A RNA polymerase II, TATA box binding protein (TBP)-associated factor [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]
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 dehydrogenase (ubiquinone) flavoprotein 1 [KO:K03942] [EC:1.6.99.3 1.6.5.3]
72900	Ndufv2; NADH dehydrogenase (ubiquinone) flavoprotein 2 [KO:K03943] [EC:1.6.99.3 1.6.5.3]
78330	Ndufv3; NADH dehydrogenase (ubiquinone) flavoprotein 3 [KO:K03944] [EC:1.6.99.3 1.6.5.3]
54405	Ndufa1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1 [KO:K03945] [EC:1.6.99.3 1.6.5.3]
17991	Ndufa2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2 [KO:K03946] [EC:1.6.99.3 1.6.5.3]
66091	Ndufa3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3 [KO:K03947] [EC:1.6.99.3 1.6.5.3]
17992	Ndufa4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4 [KO:K03948] [EC:1.6.99.3 1.6.5.3]
407790	Ndufa4l2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 [KO:K03948] [EC:1.6.99.3 1.6.5.3]
68202	Ndufa5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 5 [KO:K03949] [EC:1.6.99.3 1.6.5.3]
67130	Ndufa6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6 (B14) [KO:K03950] [EC:1.6.99.3 1.6.5.3]
66416	Ndufa7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7 (B14.5a) [KO:K03951] [EC:1.6.99.3 1.6.5.3]
68375	Ndufa8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8 [KO:K03952] [EC:1.6.99.3 1.6.5.3]
66108	Ndufa9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9 [KO:K03953] [EC:1.6.99.3 1.6.5.3]
67273	Ndufa10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10 [KO:K03954] [EC:1.6.99.3 1.6.5.3]
70316	Ndufab1; NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1 [KO:K03955] [EC:1.6.99.3 1.6.5.3]
239760	Gm4943; predicted pseudogene 4943 [KO:K03956] [EC:1.6.5.3]
69875	Ndufa11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 11 [KO:K03956] [EC:1.6.5.3]
66414	Ndufa12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12 [KO:K11352] [EC:1.6.99.3 1.6.5.3]
67184	Ndufa13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13 [KO:K11353] [EC:1.6.99.3 1.6.5.3]
68198	Ndufb2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2 [KO:K03958] [EC:1.6.99.3 1.6.5.3]
66495	Ndufb3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 3 [KO:K03959] [EC:1.6.99.3 1.6.5.3]
68194	Ndufb4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 4 [KO:K03960] [EC:1.6.99.3 1.6.5.3]
100042503	Gm3873; predicted gene 3873 [KO:K03960] [EC:1.6.99.3 1.6.5.3]
100041273	Gm3244; predicted pseudogene 3244 [KO:K03960] [EC:1.6.99.3 1.6.5.3]
66046	Ndufb5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5 [KO:K03961] [EC:1.6.99.3 1.6.5.3]
230075	Ndufb6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6 [KO:K03962] [EC:1.6.99.3 1.6.5.3]
66916	Ndufb7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7 [KO:K03963] [EC:1.6.99.3 1.6.5.3]
67264	Ndufb8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 8 [KO:K03964] [EC:1.6.99.3 1.6.5.3]
66218	Ndufb9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9 [KO:K03965] [EC:1.6.99.3 1.6.5.3]
68342	Ndufb10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10 [KO:K03966] [EC:1.6.99.3 1.6.5.3]
104130	Ndufb11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11 [KO:K11351] [EC:1.6.99.3 1.6.5.3]
227197	Ndufs1; NADH dehydrogenase (ubiquinone) Fe-S protein 1 [KO:K03934] [EC:1.6.99.3 1.6.5.3]
226646	Ndufs2; NADH dehydrogenase (ubiquinone) Fe-S protein 2 [KO:K03935] [EC:1.6.99.3 1.6.5.3]
68349	Ndufs3; NADH dehydrogenase (ubiquinone) Fe-S protein 3 [KO:K03936] [EC:1.6.99.3 1.6.5.3]
17993	Ndufs4; NADH dehydrogenase (ubiquinone) Fe-S protein 4 [KO:K03937] [EC:1.6.99.3 1.6.5.3]
595136	Ndufs5; NADH dehydrogenase (ubiquinone) Fe-S protein 5 [KO:K03938] [EC:1.6.99.3 1.6.5.3]
100504968	NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like; K03938 NADH dehydrogenase (ubiquinone) Fe-S protein 5 [EC:1.6.5.3 1.6.99.3] [KO:K03938] [EC:1.6.99.3 1.6.5.3]
407785	Ndufs6; NADH dehydrogenase (ubiquinone) Fe-S protein 6 [KO:K03939] [EC:1.6.99.3 1.6.5.3]
623286	Gm6415; predicted pseudogene 6415 [KO:K03939] [EC:1.6.99.3 1.6.5.3]
631040	NADH dehydrogenase [ubiquinone] iron-sulfur protein 6; K03939 NADH dehydrogenase (ubiquinone) Fe-S protein 6 [EC:1.6.5.3 1.6.99.3] [KO:K03939] [EC:1.6.99.3 1.6.5.3]
75406	Ndufs7; NADH dehydrogenase (ubiquinone) Fe-S protein 7 [KO:K03940] [EC:1.6.99.3 1.6.5.3]
225887	Ndufs8; NADH dehydrogenase (ubiquinone) Fe-S protein 8 [KO:K03941] [EC:1.6.99.3 1.6.5.3]
66377	Ndufc1; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1 [KO:K03967] [EC:1.6.99.3 1.6.5.3]
68197	Ndufc2; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2 [KO:K03968] [EC:1.6.99.3 1.6.5.3]
635087	NADH dehydrogenase [ubiquinone] 1 subunit C2-like; K03968 NADH dehydrogenase (ubiquinone) 1 subcomplex unknown 2 [EC:1.6.5.3 1.6.99.3] [KO:K03968] [EC:1.6.99.3 1.6.5.3]
675851	NADH dehydrogenase [ubiquinone] 1 subunit C2-like; K03968 NADH dehydrogenase (ubiquinone) 1 subcomplex unknown 2 [EC:1.6.5.3 1.6.99.3] [KO:K03968] [EC:1.6.99.3 1.6.5.3]
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:1.10.2.2]
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:1.9.3.1]
17709	COX2; cytochrome c oxidase subunit II [KO:K02261]
12857	Cox4i1; cytochrome c oxidase subunit IV isoform 1 [KO:K02263]
84682	Cox4i2; cytochrome c oxidase subunit IV isoform 2 [KO:K02263]
12858	Cox5a; cytochrome c oxidase subunit Va [KO:K02264]
12859	Cox5b; cytochrome c oxidase subunit Vb [KO:K02265]
100046079	cytochrome c oxidase subunit 5B; K02265 cytochrome c oxidase subunit 5b [KO:K02265]
12861	Cox6a1; cytochrome c oxidase subunit VIa polypeptide 1 [KO:K02266]
12862	Cox6a2; cytochrome c oxidase subunit VIa polypeptide 2 [KO:K02266]
110323	Cox6b1; cytochrome c oxidase, subunit VIb polypeptide 1 [KO:K02267]
333182	Cox6b2; cytochrome c oxidase subunit VIb polypeptide 2 [KO:K02267]
12864	Cox6c; cytochrome c oxidase subunit VIc [KO:K02268]
12866	Cox7a2; cytochrome c oxidase subunit VIIa 2 [KO:K02270]
12865	Cox7a1; cytochrome c oxidase subunit VIIa 1 [KO:K02270]
20463	Cox7a2l; cytochrome c oxidase subunit VIIa polypeptide 2-like [KO:K02270]
66142	Cox7b; cytochrome c oxidase subunit VIIb [KO:K02271]
78174	Cox7b2; cytochrome c oxidase subunit VIIb2 [KO:K02271]
12867	Cox7c; cytochrome c oxidase subunit VIIc [KO:K02272]
100048613	cytochrome c oxidase subunit 7C; K02272 cytochrome c oxidase subunit 7c [KO:K02272]
12868	Cox8a; cytochrome c oxidase subunit VIIIa [KO:K02273]
12869	Cox8b; cytochrome c oxidase subunit VIIIb [KO:K02273]
75483	Cox8c; cytochrome c oxidase subunit VIIIc [KO:K02273]
11946	Atp5a1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1 [KO:K02132] [EC:3.6.3.14]
11947	Atp5b; ATP synthase, H+ transporting mitochondrial F1 complex, beta subunit [KO:K02133] [EC:3.6.3.14]
11949	Atp5c1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136] [EC:3.6.3.14]
66043	Atp5d; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134] [EC:3.6.3.14]
67126	Atp5e; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135] [EC:3.6.3.14]
17705	ATP6; ATP synthase F0 subunit 6 [KO:K02126] [EC:3.6.3.14]
11950	Atp5f1; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit B1 [KO:K02127] [EC:3.6.3.14]
11951	Atp5g1; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit c1 (subunit 9) [KO:K02128] [EC:3.6.3.14]
67942	Atp5g2; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C2 (subunit 9) [KO:K02128] [EC:3.6.3.14]
228033	Atp5g3; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit C3 (subunit 9) [KO:K02128] [EC:3.6.3.14]
71679	Atp5h; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit d [KO:K02138] [EC:3.6.3.14]
28080	Atp5o; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137] [EC:3.6.3.14]
100047429	ATP synthase subunit O; K02137 F-type H+-transporting ATPase oligomycin sensitivity conferral protein [EC:3.6.3.14] [KO:K02137] [EC:3.6.3.14]
11957	Atp5j; ATP synthase, H+ transporting, mitochondrial F0 complex, subunit F [KO:K02131] [EC:3.6.3.14]
17706	ATP8; ATP synthase F0 subunit 8 [KO:K02125] [EC:3.6.3.14]
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 [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]
14775	Gpx1; glutathione peroxidase 1 [KO:K00432] [EC:1.11.1.9]

Compound

C00025	L-Glutamate
C00076	Calcium cation
C01245	D-myo-Inositol 1,4,5-trisphosphate

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)

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)

Reference

PMID:18951640

Authors

Bossy-Wetzel E, Petrilli A, Knott AB

Title

Mutant huntingtin and mitochondrial dysfunction.

Journal

Trends Neurosci 31:609-16 (2008)

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)

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)

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)

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)

Reference

PMID:15242639

Authors

Ross CA

Title

Huntington's disease: new paths to pathogenesis.

Journal

Cell 118:4-7 (2004)

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)

Reference

PMID:11807533

Authors

Mattson MP.

Title

Accomplices to neuronal death.

Journal

Nature 415:377-9 (2002)

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)

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)

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)

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)

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)

KO pathway

ko05016

All links

Pathway (2)   
   KEGG MODULE (1)   
   BioSystems (1)   
Genome (1)   
   KEGG GENOME (1)   
Gene (189)   
   KEGG GENES (189)   
All databases (192)   

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