KEGG PATHWAY: rno05016

PATHWAY: rno05016

Entry

rno05016 Pathway

Name

Huntington's disease - Rattus norvegicus (rat)

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

rno05016

Huntington's disease

Module

rno_M00180

RNA polymerase II, eukaryotes [PATH:rno05016]

Organism

Rattus norvegicus (rat) [GN:rno]

Gene

83800	Clta; clathrin, light chain A [KO:K04644]
116561	Cltb; clathrin, light chain B [KO:K04645]
54241	Cltc; clathrin, heavy chain (Hc) [KO:K04646]
192154	Hip1; huntingtin interacting protein 1 [KO:K04559]
81637	Ap2a2; adaptor-related protein complex 2, alpha 2 subunit [KO:K11824]
308578	Ap2a1; adaptor-related protein complex 2, alpha 1 subunit [KO:K11824]
140670	Ap2b1; adaptor-related protein complex 2, beta 1 subunit [KO:K11825]
100912146	AP-1 complex subunit beta-1-like; K11825 AP-2 complex subunit beta-1 [KO:K11825]
116563	Ap2m1; adaptor-related protein complex 2, mu 1 subunit [KO:K11826]
65046	Ap2s1; adaptor-related protein complex 2, sigma 1 subunit [KO:K11827]
29424	Htt; huntingtin [KO:K04533]
303968	Ift57; intraflagellar transport 57 homolog (Chlamydomonas) [KO:K04638]
64044	Casp8; caspase 8 [KO:K04398] [EC:3.4.22.61]
25402	Casp3; caspase 3 [KO:K02187] [EC:3.4.22.56]
24418	Grm5; glutamate receptor, metabotropic 5 [KO:K04604]
81666	Gnaq; guanine nucleotide binding protein (G protein), q polypeptide [KO:K04634]
24654	Plcb1; phospholipase C, beta 1 (phosphoinositide-specific) [KO:K05858] [EC:3.1.4.11]
29322	Plcb3; phospholipase C, beta 3 (phosphatidylinositol-specific) [KO:K05858] [EC:3.1.4.11]
85240	Plcb2; phospholipase C, beta 2 [KO:K05858] [EC:3.1.4.11]
25031	Plcb4; phospholipase C, beta 4 [KO:K05858] [EC:3.1.4.11]
24408	Grin1; glutamate receptor, ionotropic, N-methyl D-aspartate 1 [KO:K05208]
24410	Grin2b; glutamate receptor, ionotropic, N-methyl D-aspartate 2B [KO:K05210]
25262	Itpr1; inositol 1,4,5-trisphosphate receptor, type 1 [KO:K04958]
29495	Dlg4; discs, large homolog 4 (Drosophila) [KO:K11828]
56083	Tgm2; transglutaminase 2, C polypeptide [KO:K05625] [EC:2.3.2.13]
83618	Rest; RE1-silencing transcription factor [KO:K09222]
363067	Sin3a; SIN3 transcription regulator homolog A (yeast) [KO:K11644]
297893	Hdac1; histone deacetylase 1 [KO:K06067] [EC:3.5.1.98]
84576	Hdac1l; histone deacetylase 1-like [KO:K06067] [EC:3.5.1.98]
84577	Hdac2; histone deacetylase 2 [KO:K06067] [EC:3.5.1.98]
363633	Polr2a; polymerase (RNA) II (DNA directed) polypeptide A [KO:K03006] [EC:2.7.7.6]
289561	Polr2b; polymerase (RNA) II (DNA directed) polypeptide B [KO:K03010] [EC:2.7.7.6]
361365	Polr2c; polymerase (RNA) II (DNA directed) polypeptide C [KO:K03011]
364834	Polr2d; polymerase (RNA) II (DNA directed) polypeptide D [KO:K03012]
690966	Polr2e; polymerase (RNA) II (DNA directed) polypeptide E [KO:K03013]
83503	Polr2f; polymerase (RNA) II (DNA directed) polypeptide F [KO:K03014]
117017	Polr2g; polymerase (RNA) II (DNA directed) polypeptide G [KO:K03015]
291795	RGD1565904; similar to polymerase (RNA) II (DNA directed) polypeptide H [KO:K03016]
498109	Polr2h; polymerase (RNA) II (DNA directed) polypeptide H [KO:K03016]
100912534	DNA-directed RNA polymerases I; K03016 DNA-directed RNA polymerases I, II, and III subunit RPABC3 [KO:K03016]
292778	Polr2i; polymerase (RNA) II (DNA directed) polypeptide I [KO:K03017]
100911822	DNA-directed RNA polymerase II subunit RPB9-like; K03017 DNA-directed RNA polymerase II subunit RPB9 [KO:K03017]
681193	similar to DNA-directed RNA polymerase II 7.6 kDa polypeptide (RPB10) (RPB7.6) (RPABC5); K03007 DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
502374	Polr2l; polymerase (RNA) II (DNA directed) polypeptide L [KO:K03007]
100911840	DNA-directed RNA polymerases I; K03007 DNA-directed RNA polymerases I, II, and III subunit RPABC5 [KO:K03007]
288588	Polr2j; polymerase (RNA) II (DNA directed) polypeptide J [KO:K03008]
100363176	mCG145114-like; K03009 DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
100361574	mCG145114-like; K03009 DNA-directed RNA polymerases I, II, and III subunit RPABC4 [KO:K03009]
24225	Bdnf; brain-derived neurotrophic factor [KO:K04355]
29167	Dctn1; dynactin 1 [KO:K04648]
299850	Dctn2; dynactin 2 (p50) [KO:K10424]
84428	Dctn4; dynactin 4 (p62) [KO:K10426]
29430	Hap1; huntingtin-associated protein 1 [KO:K04647]
171339	Dnah1; dynein, axonemal, heavy chain 1 [KO:K10408]
303242	Dnah2; dynein, axonemal, heavy chain 2 [KO:K10408]
117249	Dnah3; dynein, axonemal, heavy chain 3 [KO:K10408]
298524	Dnali1; dynein, axonemal, light intermediate chain 1 [KO:K10410]
500442	Dnai1; dynein, axonemal, intermediate chain 1 [KO:K10409]
360654	Dnai2; dynein, axonemal, intermediate chain 2 [KO:K11143]
685664	Dnal1; dynein, axonemal, light chain 1 [KO:K10411]
300078	Dnal4; dynein, axonemal, light chain 4 [KO:K10412]
294376	RGD1559781; similar to Dynein, axonemal, light chain 4 [KO:K10412]
54244	Crebbp; CREB binding protein [KO:K04498] [EC:2.3.1.48]
170915	Ep300; E1A binding protein p300 [KO:K04498] [EC:2.3.1.48]
291773	RGD1562997; similar to Transcription initiation factor TFIID 105 kDa subunit (TAFII-105) [KO:K03129]
117526	Tbp; TATA box binding protein [KO:K03120]
680050	Tbpl2; TATA box binding protein-like 2 [KO:K03120]
689030	Tbpl1; TBP-like 1 [KO:K03120]
24790	Sp1; Sp1 transcription factor [KO:K04684]
81646	Creb1; cAMP responsive element binding protein 1 [KO:K05870]
310616	Creb3l4; cAMP responsive element binding protein 3-like 4 [KO:K09048]
362165	Creb3l1; cAMP responsive element binding protein 3-like 1 [KO:K09048]
362339	Creb3l2; cAMP responsive element binding protein 3-like 2 [KO:K09048]
314638	Creb3l3; cAMP responsive element binding protein 3-like 3 [KO:K09048]
500131	Creb5; cAMP responsive element binding protein 5 [KO:K09047]
83516	Ppargc1a; peroxisome proliferator-activated receptor gamma, coactivator 1 alpha [KO:K07202]
25664	Pparg; peroxisome proliferator-activated receptor gamma [KO:K08530]
83474	Tfam; transcription factor A, mitochondrial [KO:K11830]
312195	Nrf1; nuclear respiratory factor 1 [KO:K11831]
24786	Sod1; superoxide dismutase 1, soluble [KO:K04565] [EC:1.15.1.1]
24787	Sod2; superoxide dismutase 2, mitochondrial [KO:K04564] [EC:1.15.1.1]
24860	Ucp1; uncoupling protein 1 (mitochondrial, proton carrier) [KO:K08769]
24842	Tp53; tumor protein p53 [KO:K04451]
24887	Bax; Bcl2-associated X protein [KO:K02159]
681314	similar to BAX protein; K02159 apoptosis regulator BAX [KO:K02159]
317673	Bbc3; Bcl-2 binding component 3 [KO:K10132]
293655	Ndufv1; NADH dehydrogenase (ubiquinone) flavoprotein 1 [KO:K03942] [EC:1.6.99.3 1.6.5.3]
81728	Ndufv2; NADH dehydrogenase (ubiquinone) flavoprotein 2 [KO:K03943] [EC:1.6.99.3 1.6.5.3]
64539	Ndufv3; NADH dehydrogenase (ubiquinone) flavoprotein 3 [KO:K03944] [EC:1.6.99.3 1.6.5.3]
363441	Ndufa1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 1 [KO:K03945] [EC:1.6.99.3 1.6.5.3]
291660	Ndufa2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 2 [KO:K03946] [EC:1.6.99.3 1.6.5.3]
691015	similar to NADH dehydrogenase (ubiquinone) 1 alpha subcomplex; K03946 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 2 [EC:1.6.5.3 1.6.99.3] [KO:K03946] [EC:1.6.99.3 1.6.5.3]
680288	similar to NADH-ubiquinone oxidoreductase B9 subunit (Complex I-B9) (CI-B9); K03947 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 3 [EC:1.6.5.3 1.6.99.3] [KO:K03947] [EC:1.6.99.3 1.6.5.3]
691001	Ndufa3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 3 [KO:K03947] [EC:1.6.99.3 1.6.5.3]
684509	similar to NADH-ubiquinone oxidoreductase B9 subunit (Complex I-B9) (CI-B9); K03947 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 3 [EC:1.6.5.3 1.6.99.3] [KO:K03947] [EC:1.6.99.3 1.6.5.3]
681024	Ndufa4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4 [KO:K03948] [EC:1.6.99.3 1.6.5.3]
100362331	Ndufa4l2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 4-like 2 [KO:K03948] [EC:1.6.99.3 1.6.5.3]
25488	Ndufa5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 5 [KO:K03949] [EC:1.6.99.3 1.6.5.3]
315167	Ndufa6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 6 (B14) [KO:K03950] [EC:1.6.99.3 1.6.5.3]
299643	Ndufa7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7 [KO:K03951] [EC:1.6.99.3 1.6.5.3]
366074	Ndufa7l; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 7-like [KO:K03951] [EC:1.6.99.3 1.6.5.3]
296658	Ndufa8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 8 [KO:K03952] [EC:1.6.99.3 1.6.5.3]
362440	Ndufa9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 9 [KO:K03953] [EC:1.6.99.3 1.6.5.3]
316632	Ndufa10l1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10-like 1 [KO:K03954] [EC:1.6.99.3 1.6.5.3]
678759	Ndufa10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 10 [KO:K03954] [EC:1.6.99.3 1.6.5.3]
293453	Ndufab1; NADH dehydrogenase (ubiquinone) 1, alpha/beta subcomplex, 1 [KO:K03955] [EC:1.6.99.3 1.6.5.3]
683884	similar to Acyl carrier protein; K03955 NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1 [EC:1.6.5.3 1.6.99.3] [KO:K03955] [EC:1.6.99.3 1.6.5.3]
301123	Ndufa11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 11 [KO:K03956] [EC:1.6.5.3]
299739	Ndufa12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 12 [KO:K11352] [EC:1.6.99.3 1.6.5.3]
314759	Ndufa13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex, 13 [KO:K11353] [EC:1.6.99.3 1.6.5.3]
100911483	NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13-like; K11353 NADH dehydrogenase (ubiquinone) 1 alpha subcomplex 13 [EC:1.6.5.3 1.6.99.3] [KO:K11353] [EC:1.6.99.3 1.6.5.3]
362344	Ndufb2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 2 [KO:K03958] [EC:1.6.99.3 1.6.5.3]
301427	Ndufb3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 3 [KO:K03959] [EC:1.6.99.3 1.6.5.3]
288088	Ndufb4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 4 [KO:K03960] [EC:1.6.99.3 1.6.5.3]
690550	similar to NADH dehydrogenase (ubiquinone) 1 beta subcomplex; K03960 NADH dehydrogenase (ubiquinone) 1 beta subcomplex 4 [EC:1.6.5.3 1.6.99.3] [KO:K03960] [EC:1.6.99.3 1.6.5.3]
691675	similar to NADH dehydrogenase (ubiquinone) 1 beta subcomplex; K03960 NADH dehydrogenase (ubiquinone) 1 beta subcomplex 4 [EC:1.6.5.3 1.6.99.3] [KO:K03960] [EC:1.6.99.3 1.6.5.3]
501886	Ndufb4l1; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 4-like 1 [KO:K03960] [EC:1.6.99.3 1.6.5.3]
688963	similar to NADH dehydrogenase (ubiquinone) 1 beta subcomplex; K03960 NADH dehydrogenase (ubiquinone) 1 beta subcomplex 4 [EC:1.6.5.3 1.6.99.3] [KO:K03960] [EC:1.6.99.3 1.6.5.3]
499131	RGD1560088; similar to NADH:ubiquinone oxidoreductase B15 subunit [KO:K03960] [EC:1.6.99.3 1.6.5.3]
294964	Ndufb5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 5 [KO:K03961] [EC:1.6.99.3 1.6.5.3]
297990	Ndufb6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 6 [KO:K03962] [EC:1.6.99.3 1.6.5.3]
361385	Ndufb7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 7 [KO:K03963] [EC:1.6.99.3 1.6.5.3]
293991	Ndufb8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex 8 [KO:K03964] [EC:1.6.99.3 1.6.5.3]
299954	Ndufb9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 9 [KO:K03965] [EC:1.6.99.3 1.6.5.3]
681418	Ndufb10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 10 [KO:K03966] [EC:1.6.99.3 1.6.5.3]
299310	Ndufb11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex, 11 [KO:K11351] [EC:1.6.99.3 1.6.5.3]
301458	Ndufs1; NADH dehydrogenase (ubiquinone) Fe-S protein 1 [KO:K03934] [EC:1.6.99.3 1.6.5.3]
289218	Ndufs2; NADH dehydrogenase (ubiquinone) Fe-S protein 2 [KO:K03935] [EC:1.6.99.3 1.6.5.3]
295923	Ndufs3; NADH dehydrogenase (ubiquinone) Fe-S protein 3 [KO:K03936] [EC:1.6.99.3 1.6.5.3]
499529	Ndufs4; NADH dehydrogenase (ubiquinone) Fe-S protein 4 [KO:K03937] [EC:1.6.99.3 1.6.5.3]
362588	Ndufs5; NADH dehydrogenase (ubiquinone) Fe-S protein 5 [KO:K03938] [EC:1.6.99.3 1.6.5.3]
100363268	rCG31129-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]
29478	Ndufs6; NADH dehydrogenase (ubiquinone) Fe-S protein 6 [KO:K03939] [EC:1.6.99.3 1.6.5.3]
679739	NADH dehydrogenase (ubiquinone) Fe-S 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]
100912599	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]
362837	Ndufs7; NADH dehydrogenase (ubiquinone) Fe-S protein 7 [KO:K03940] [EC:1.6.99.3 1.6.5.3]
293652	Ndufs8; NADH dehydrogenase (ubiquinone) Fe-S protein 8 [KO:K03941] [EC:1.6.99.3 1.6.5.3]
689938	Ndufc1; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 1 [KO:K03967] [EC:1.6.99.3 1.6.5.3]
293130	Ndufc2; NADH dehydrogenase (ubiquinone) 1, subcomplex unknown, 2 [KO:K03968] [EC:1.6.99.3 1.6.5.3]
157074	Sdha; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234] [EC:1.3.5.1]
298596	Sdhb; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235] [EC:1.3.5.1]
289217	Sdhc; succinate dehydrogenase complex, subunit C, integral membrane protein [KO:K00236]
363061	Sdhd; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237]
291103	Uqcrfs1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411] [EC:1.10.2.2]
26192	CYTB; cytochrome b [KO:K00412]
300047	Cyc1; cytochrome c-1 [KO:K00413]
689099	hypothetical protein LOC689099; K00413 ubiquinol-cytochrome c reductase cytochrome c1 subunit [KO:K00413]
301011	Uqcrc1; ubiquinol-cytochrome c reductase core protein I [KO:K00414]
293448	Uqcrc2; ubiquinol cytochrome c reductase core protein 2 [KO:K00415]
366448	Uqcrh; ubiquinol-cytochrome c reductase hinge protein [KO:K00416]
362897	Uqcrb; ubiquinol-cytochrome c reductase binding protein [KO:K00417]
685596	similar to ubiquinol-cytochrome c reductase binding protein; K00417 ubiquinol-cytochrome c reductase subunit 7 [KO:K00417]
692017	similar to ubiquinol-cytochrome c reductase binding protein; K00417 ubiquinol-cytochrome c reductase subunit 7 [KO:K00417]
497902	Uqcrq; ubiquinol-cytochrome c reductase, complex III subunit VII [KO:K00418]
685322	Ucrc; similar to ubiquinol-cytochrome c reductase complex 7.2kDa protein isoform b [KO:K00419]
100361126	Uqcr10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419]
686442	similar to ubiquinol-cytochrome c reductase subunit; K00420 ubiquinol-cytochrome c reductase subunit 10 [KO:K00420]
690848	Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420]
26204	COX3; cytochrome c oxidase subunit 3 [KO:K02262]
26195	COX1; cytochrome c oxidase subunit 1 [KO:K02256] [EC:1.9.3.1]
26198	COX2; COXII [KO:K02261]
84683	Cox4i2; cytochrome c oxidase subunit IV isoform 2 (lung) [KO:K02263]
29445	Cox4i1; cytochrome c oxidase subunit IV isoform 1 [KO:K02263]
252934	Cox5a; cytochrome c oxidase, subunit Va [KO:K02264]
94194	Cox5b; cytochrome c oxidase subunit Vb [KO:K02265]
25278	Cox6a2; cytochrome c oxidase subunit VIa polypeptide 2 [KO:K02266]
25282	Cox6a1; cytochrome c oxidase, subunit VIa, polypeptide 1 [KO:K02266]
688869	Cox6b1; similar to cytochrome c oxidase, subunit VIb polypeptide 1 [KO:K02267]
654441	Cox6b2; cytochrome c oxidase subunit VIb polypeptide 2 [KO:K02267]
681754	similar to cytochrome c oxidase; K02267 cytochrome c oxidase subunit 6b [KO:K02267]
54322	Cox6c; cytochrome c oxidase, subunit VIc [KO:K02268]
100365711	cytochrome c oxidase; K02268 cytochrome c oxidase subunit 6c [KO:K02268]
29507	Cox7a2; cytochrome c oxidase subunit VIIa polypeptide 2 [KO:K02270]
298762	Cox7a2l; cytochrome c oxidase subunit VIIa polypeptide 2 like [KO:K02270]
688386	Cox7a2l2; cytochrome c oxidase subunit VIIa polypeptide 2-like 2 [KO:K02270]
687508	similar to Cytochrome c oxidase polypeptide VIIa-heart; K02270 cytochrome c oxidase subunit 7a [KO:K02270]
100910772	cytochrome c oxidase subunit 7A1; K02270 cytochrome c oxidase subunit 7a [KO:K02270]
303393	Cox7b; cytochrome c oxidase subunit VIIb [KO:K02271]
100361960	Cox7b2; cytochrome c oxidase subunit VIIb2 [KO:K02271]
100188937	Cox7c; cytochrome c oxidase, subunit VIIc [KO:K02272]
25250	Cox8b; cytochrome c oxidase, subunit VIIIb [KO:K02273]
360229	Cox8c; cytochrome c oxidase, subunit VIIIc [KO:K02273]
171335	Cox8a; cytochrome c oxidase subunit VIIIa [KO:K02273]
65262	Atp5a1; ATP synthase, H+ transporting, mitochondrial F1 complex, alpha subunit 1, cardiac muscle [KO:K02132] [EC:3.6.3.14]
171374	Atp5b; ATP synthase, H+ transporting, mitochondrial F1 complex, beta polypeptide [KO:K02133] [EC:3.6.3.14]
116550	Atp5c1; ATP synthase, H+ transporting, mitochondrial F1 complex, gamma polypeptide 1 [KO:K02136] [EC:3.6.3.14]
245965	Atp5d; ATP synthase, H+ transporting, mitochondrial F1 complex, delta subunit [KO:K02134] [EC:3.6.3.14]
245958	Atp5e; ATP synthase, H+ transporting, mitochondrial F1 complex, epsilon subunit [KO:K02135] [EC:3.6.3.14]
26197	ATP6; ATPase subunit 6 [KO:K02126] [EC:3.6.3.14]
171375	Atp5f1; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit B1 [KO:K02127] [EC:3.6.3.14]
100911417	ATP synthase subunit b; K02127 F-type H+-transporting ATPase subunit b [EC:3.6.3.14] [KO:K02127] [EC:3.6.3.14]
171082	Atp5g2; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C2 (subunit 9) [KO:K02128] [EC:3.6.3.14]
114630	Atp5g3; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C3 (subunit 9) [KO:K02128] [EC:3.6.3.14]
29754	Atp5g1; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit C1 (subunit 9) [KO:K02128] [EC:3.6.3.14]
689961	similar to ATP synthase lipid-binding protein; K02128 F-type H+-transporting ATPase subunit c [EC:3.6.3.14] [KO:K02128] [EC:3.6.3.14]
366614	RGD1559629; similar to H+ ATP synthase [KO:K02128] [EC:3.6.3.14]
306478	Atp5hl1; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d-like 1 [KO:K02138] [EC:3.6.3.14]
641434	Atp5h; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit d [KO:K02138] [EC:3.6.3.14]
192241	Atp5o; ATP synthase, H+ transporting, mitochondrial F1 complex, O subunit [KO:K02137] [EC:3.6.3.14]
94271	Atp5j; ATP synthase, H+ transporting, mitochondrial Fo complex, subunit F6 [KO:K02131] [EC:3.6.3.14]
26196	ATP8; ATPase subunit 8 [KO:K02125] [EC:3.6.3.14]
83529	Vdac1; voltage-dependent anion channel 1 [KO:K05862]
679923	similar to voltage-dependent anion channel 1; K05862 voltage-dependent anion channel protein 1 [KO:K05862]
683062	similar to voltage-dependent anion channel 1; K05862 voltage-dependent anion channel protein 1 [KO:K05862]
83531	Vdac2; voltage-dependent anion channel 2 [KO:K15040]
83532	Vdac3; voltage-dependent anion channel 3 [KO:K15041]
25176	Slc25a5; solute carrier family 25 (mitochondrial carrier [KO:K05863]
85333	Slc25a4; solute carrier family 25 (mitochondrial carrier [KO:K05863]
689108	Slc25a31; solute carrier family 25 (mitochondrial carrier [KO:K05863]
282819	Ppif; peptidylprolyl isomerase F [KO:K09565] [EC:5.2.1.8]
25309	Cycs; cytochrome c, somatic [KO:K08738]
25310	Cyct; cytochrome c, testis [KO:K08738]
679794	similar to Cytochrome c; K08738 cytochrome c [KO:K08738]
689459	similar to Cytochrome c; K08738 cytochrome c [KO:K08738]
690675	similar to Cytochrome c; K08738 cytochrome c [KO:K08738]
100363502	cytochrome c; K08738 cytochrome c [KO:K08738]
78963	Apaf1; apoptotic peptidase activating factor 1 [KO:K02084]
58918	Casp9; caspase 9, apoptosis-related cysteine peptidase [KO:K04399] [EC:3.4.22.62]
24404	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 (219)   
   KEGG GENES (219)   
All databases (222)   

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