KEGG   PATHWAY: ko05016
Entry
ko05016                     Pathway                                
Name
Huntington disease
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
ko05016  Huntington disease
ko05016

Disease
H00059  Huntington disease
Orthology
K00234  SDHA, SDH1; succinate dehydrogenase (ubiquinone) flavoprotein subunit [EC:1.3.5.1]
K00235  SDHB, SDH2; succinate dehydrogenase (ubiquinone) iron-sulfur subunit [EC:1.3.5.1]
K00236  SDHC, SDH3; succinate dehydrogenase (ubiquinone) cytochrome b560 subunit
K00237  SDHD, SDH4; succinate dehydrogenase (ubiquinone) membrane anchor subunit
K00411  UQCRFS1, RIP1, petA; ubiquinol-cytochrome c reductase iron-sulfur subunit [EC:7.1.1.8]
K00412  CYTB, petB; ubiquinol-cytochrome c reductase cytochrome b subunit
K00413  CYC1, CYT1, petC; ubiquinol-cytochrome c reductase cytochrome c1 subunit
K00414  QCR1, UQCRC1; ubiquinol-cytochrome c reductase core subunit 1
K00415  QCR2, UQCRC2; ubiquinol-cytochrome c reductase core subunit 2
K00416  QCR6, UQCRH; ubiquinol-cytochrome c reductase subunit 6
K00417  QCR7, UQCRB; ubiquinol-cytochrome c reductase subunit 7
K00418  QCR8, UQCRQ; ubiquinol-cytochrome c reductase subunit 8
K00419  QCR9, UCRC; ubiquinol-cytochrome c reductase subunit 9
K00420  QCR10, UQCR; ubiquinol-cytochrome c reductase subunit 10
K00432  gpx, btuE, bsaA; glutathione peroxidase [EC:1.11.1.9]
K00914  PIK3C3, VPS34; phosphatidylinositol 3-kinase [EC:2.7.1.137]
K02084  APAF1; apoptotic protease-activating factor
K02125  ATPeF08, MTATP8, ATP8; F-type H+-transporting ATPase subunit 8
K02126  ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
K02127  ATPeF0B, ATP5F1, ATP4; F-type H+-transporting ATPase subunit b
K02128  ATPeF0C, ATP5G, ATP9; F-type H+-transporting ATPase subunit c
K02131  ATPeF0F6, ATP5J; F-type H+-transporting ATPase subunit 6
K02132  ATPeF1A, ATP5A1, ATP1; F-type H+-transporting ATPase subunit alpha
K02133  ATPeF1B, ATP5B, ATP2; F-type H+-transporting ATPase subunit beta [EC:7.1.2.2]
K02134  ATPeF1D, ATP5D, ATP16; F-type H+-transporting ATPase subunit delta
K02135  ATPeF1E, ATP5E, ATP15; F-type H+-transporting ATPase subunit epsilon
K02136  ATPeF1G, ATP5C1, ATP3; F-type H+-transporting ATPase subunit gamma
K02137  ATPeF0O, ATP5O, ATP5; F-type H+-transporting ATPase subunit O
K02138  ATPeF0D, ATP5H, ATP7; F-type H+-transporting ATPase subunit d
K02159  BAX; apoptosis regulator BAX
K02187  CASP3; caspase 3 [EC:3.4.22.56]
K02256  COX1; cytochrome c oxidase subunit 1 [EC:7.1.1.9]
K02261  COX2; cytochrome c oxidase subunit 2
K02262  COX3; cytochrome c oxidase subunit 3
K02263  COX4; cytochrome c oxidase subunit 4
K02264  COX5A; cytochrome c oxidase subunit 5a
K02265  COX5B; cytochrome c oxidase subunit 5b
K02266  COX6A; cytochrome c oxidase subunit 6a
K02267  COX6B; cytochrome c oxidase subunit 6b
K02268  COX6C; cytochrome c oxidase subunit 6c
K02270  COX7A; cytochrome c oxidase subunit 7a
K02271  COX7B; cytochrome c oxidase subunit 7b
K02272  COX7C; cytochrome c oxidase subunit 7c
K02273  COX8; cytochrome c oxidase subunit 8
K02725  PSMA1; 20S proteasome subunit alpha 6 [EC:3.4.25.1]
K02726  PSMA2; 20S proteasome subunit alpha 2 [EC:3.4.25.1]
K02727  PSMA3; 20S proteasome subunit alpha 7 [EC:3.4.25.1]
K02728  PSMA4; 20S proteasome subunit alpha 3 [EC:3.4.25.1]
K02729  PSMA5; 20S proteasome subunit alpha 5 [EC:3.4.25.1]
K02730  PSMA6; 20S proteasome subunit alpha 1 [EC:3.4.25.1]
K02731  PSMA7; 20S proteasome subunit alpha 4 [EC:3.4.25.1]
K02732  PSMB1; 20S proteasome subunit beta 6 [EC:3.4.25.1]
K02734  PSMB2; 20S proteasome subunit beta 4 [EC:3.4.25.1]
K02735  PSMB3; 20S proteasome subunit beta 3 [EC:3.4.25.1]
K02736  PSMB4; 20S proteasome subunit beta 7 [EC:3.4.25.1]
K02737  PSMB5; 20S proteasome subunit beta 5 [EC:3.4.25.1]
K02738  PSMB6; 20S proteasome subunit beta 1 [EC:3.4.25.1]
K02739  PSMB7; 20S proteasome subunit beta 2 [EC:3.4.25.1]
K03006  RPB1, POLR2A; DNA-directed RNA polymerase II subunit RPB1 [EC:2.7.7.6]
K03007  RPABC5, RPB10, POLR2L; DNA-directed RNA polymerases I, II, and III subunit RPABC5
K03008  RPB11, POLR2J; DNA-directed RNA polymerase II subunit RPB11
K03009  RPABC4, RPB12, POLR2K; DNA-directed RNA polymerases I, II, and III subunit RPABC4
K03010  RPB2, POLR2B; DNA-directed RNA polymerase II subunit RPB2 [EC:2.7.7.6]
K03011  RPB3, POLR2C; DNA-directed RNA polymerase II subunit RPB3
K03012  RPB4, POLR2D; DNA-directed RNA polymerase II subunit RPB4
K03013  RPABC1, RPB5, POLR2E; DNA-directed RNA polymerases I, II, and III subunit RPABC1
K03014  RPABC2, RPB6, POLR2F; DNA-directed RNA polymerases I, II, and III subunit RPABC2
K03015  RPB7, POLR2G; DNA-directed RNA polymerase II subunit RPB7
K03016  RPABC3, RPB8, POLR2H; DNA-directed RNA polymerases I, II, and III subunit RPABC3
K03017  RPB9, POLR2I; DNA-directed RNA polymerase II subunit RPB9
K03028  PSMD2, RPN1; 26S proteasome regulatory subunit N1
K03029  PSMD4, RPN10; 26S proteasome regulatory subunit N10
K03030  PSMD14, RPN11, POH1; 26S proteasome regulatory subunit N11
K03031  PSMD8, RPN12; 26S proteasome regulatory subunit N12
K03032  PSMD1, RPN2; 26S proteasome regulatory subunit N2
K03033  PSMD3, RPN3; 26S proteasome regulatory subunit N3
K03035  PSMD12, RPN5; 26S proteasome regulatory subunit N5
K03036  PSMD11, RPN6; 26S proteasome regulatory subunit N6
K03037  PSMD6, RPN7; 26S proteasome regulatory subunit N7
K03038  PSMD7, RPN8; 26S proteasome regulatory subunit N8
K03039  PSMD13, RPN9; 26S proteasome regulatory subunit N9
K03061  PSMC2, RPT1; 26S proteasome regulatory subunit T1
K03062  PSMC1, RPT2; 26S proteasome regulatory subunit T2
K03063  PSMC4, RPT3; 26S proteasome regulatory subunit T3
K03064  PSMC6, RPT4; 26S proteasome regulatory subunit T4
K03065  PSMC3, RPT5; 26S proteasome regulatory subunit T5
K03066  PSMC5, RPT6; 26S proteasome regulatory subunit T6
K03120  TBP; transcription initiation factor TFIID TATA-box-binding protein
K03129  TAF4; transcription initiation factor TFIID subunit 4
K03173  TRAF2; TNF receptor-associated factor 2 [EC:2.3.2.27]
K03878  ND1; NADH-ubiquinone oxidoreductase chain 1 [EC:7.1.1.2]
K03879  ND2; NADH-ubiquinone oxidoreductase chain 2 [EC:7.1.1.2]
K03880  ND3; NADH-ubiquinone oxidoreductase chain 3 [EC:7.1.1.2]
K03881  ND4; NADH-ubiquinone oxidoreductase chain 4 [EC:7.1.1.2]
K03882  ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2]
K03883  ND5; NADH-ubiquinone oxidoreductase chain 5 [EC:7.1.1.2]
K03884  ND6; NADH-ubiquinone oxidoreductase chain 6 [EC:7.1.1.2]
K03934  NDUFS1; NADH dehydrogenase (ubiquinone) Fe-S protein 1 [EC:7.1.1.2]
K03935  NDUFS2; NADH dehydrogenase (ubiquinone) Fe-S protein 2 [EC:7.1.1.2]
K03936  NDUFS3; NADH dehydrogenase (ubiquinone) Fe-S protein 3 [EC:7.1.1.2]
K03937  NDUFS4; NADH dehydrogenase (ubiquinone) Fe-S protein 4
K03938  NDUFS5; NADH dehydrogenase (ubiquinone) Fe-S protein 5
K03939  NDUFS6; NADH dehydrogenase (ubiquinone) Fe-S protein 6
K03940  NDUFS7; NADH dehydrogenase (ubiquinone) Fe-S protein 7 [EC:7.1.1.2]
K03941  NDUFS8; NADH dehydrogenase (ubiquinone) Fe-S protein 8 [EC:7.1.1.2]
K03942  NDUFV1; NADH dehydrogenase (ubiquinone) flavoprotein 1 [EC:7.1.1.2]
K03943  NDUFV2; NADH dehydrogenase (ubiquinone) flavoprotein 2 [EC:7.1.1.2]
K03944  NDUFV3; NADH dehydrogenase (ubiquinone) flavoprotein 3
K03945  NDUFA1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 1
K03946  NDUFA2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 2
K03947  NDUFA3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 3
K03948  NDUFA4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4
K03949  NDUFA5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 5
K03950  NDUFA6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 6
K03951  NDUFA7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 7
K03952  NDUFA8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 8
K03953  NDUFA9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 9
K03954  NDUFA10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 10
K03955  NDUFAB1; NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1, acyl-carrier protein
K03956  NDUFA11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 11
K03957  NDUFB1; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 1
K03958  NDUFB2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 2
K03959  NDUFB3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 3
K03960  NDUFB4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 4
K03961  NDUFB5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 5
K03962  NDUFB6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6
K03963  NDUFB7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7
K03964  NDUFB8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8
K03965  NDUFB9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9
K03966  NDUFB10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10
K03967  NDUFC1; NADH dehydrogenase (ubiquinone) 1 subunit C1
K03968  NDUFC2; NADH dehydrogenase (ubiquinone) 1 subunit C2
K04355  BDNF; brain-derived neurotrophic factor
K04398  CASP8; caspase 8 [EC:3.4.22.61]
K04399  CASP9; caspase 9 [EC:3.4.22.62]
K04418  MAP3K10, MLK2; mitogen-activated protein kinase kinase kinase 10 [EC:2.7.11.25]
K04426  MAP3K5, ASK1; mitogen-activated protein kinase kinase kinase 5 [EC:2.7.11.25]
K04431  MAP2K7, MKK7; mitogen-activated protein kinase kinase 7 [EC:2.7.12.2]
K04440  JNK; mitogen-activated protein kinase 8/9/10 (c-Jun N-terminal kinase) [EC:2.7.11.24]
K04451  TP53, P53; tumor protein p53
K04498  EP300, CREBBP, KAT3; E1A/CREB-binding protein [EC:2.3.1.48]
K04533  HD; huntingtin
K04559  HIP1; huntingtin interacting protein 1
K04560  STX1A; syntaxin 1A
K04564  SOD2; superoxide dismutase, Fe-Mn family [EC:1.15.1.1]
K04565  SOD1; superoxide dismutase, Cu-Zn family [EC:1.15.1.1]
K04604  GRM5; metabotropic glutamate receptor 5
K04634  GNAQ; guanine nucleotide-binding protein G(q) subunit alpha
K04638  IFT57, HIPPI, ESRRBL1; intraflagellar transport protein 57
K04644  CLTA, LCA; clathrin light chain A
K04645  CLTB, LCB; clathrin light chain B
K04646  CLTC; clathrin heavy chain
K04647  HAP1; huntingtin-associated protein 1
K04648  DCTN1; dynactin 1
K04684  SP1; transcription factor Sp1
K04849  CACNA1B, CAV2.2; voltage-dependent calcium channel N type alpha-1B
K04958  ITPR1; inositol 1,4,5-triphosphate receptor type 1
K05003  KCNJ10, KIR4.1; potassium inwardly-rectifying channel subfamily J member 10
K05197  GRIA1; glutamate receptor 1
K05198  GRIA2; glutamate receptor 2
K05199  GRIA3; glutamate receptor 3
K05200  GRIA4; glutamate receptor 4
K05208  GRIN1; glutamate receptor ionotropic, NMDA 1
K05210  GRIN2B; glutamate receptor ionotropic, NMDA 2B
K05613  SLC1A2, EAAT2; solute carrier family 1 (glial high affinity glutamate transporter), member 2
K05614  SLC1A3, EAAT1; solute carrier family 1 (glial high affinity glutamate transporter), member 3
K05625  TGM2; transglutaminase 2 [EC:2.3.2.13]
K05858  PLCB; phosphatidylinositol phospholipase C, beta [EC:3.1.4.11]
K05862  VDAC1; voltage-dependent anion channel protein 1
K05863  SLC25A4S, ANT; solute carrier family 25 (mitochondrial adenine nucleotide translocator), member 4/5/6/31
K05870  CREB1; cyclic AMP-responsive element-binding protein 1
K06067  HDAC1_2; histone deacetylase 1/2 [EC:3.5.1.98]
K06691  RPN13; 26S proteasome regulatory subunit N13
K06693  PSMD9, RPN4; 26S proteasome regulatory subunit N4
K07202  PPARGC1A, PGC1A; alpha peroxisome proliferator-activated receptor gamma coactivator 1-alpha
K07203  MTOR, FRAP, TOR; serine/threonine-protein kinase mTOR [EC:2.7.11.1]
K07374  TUBA; tubulin alpha
K07375  TUBB; tubulin beta
K08269  ULK2, ATG1; serine/threonine-protein kinase ULK2 [EC:2.7.11.1]
K08331  ATG13; autophagy-related protein 13
K08333  PIK3R4, VPS15; phosphoinositide-3-kinase, regulatory subunit 4 [EC:2.7.11.1]
K08334  BECN, VPS30, ATG6; beclin
K08530  NR1C3, PPARG; peroxisome proliferator-activated receptor gamma
K08738  CYC; cytochrome c
K08769  UCP1, SLC25A7; solute carrier family 25 (mitochondrial uncoupling protein), member 7
K08852  ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [EC:2.7.11.1 3.1.26.-]
K09047  CREB5, CREBPA; cyclic AMP-responsive element-binding protein 5
K09048  CREB3; cyclic AMP-responsive element-binding protein 3
K09222  REST; RE1-silencing transcription factor
K09565  PPIF; peptidyl-prolyl isomerase F (cyclophilin D) [EC:5.2.1.8]
K10132  BBC3, PUMA; Bcl-2-binding component 3
K10396  KIF5; kinesin family member 5 (plus-end-directed kinesin ATPase) [EC:5.6.1.3]
K10407  KLC; kinesin light chain
K10408  DNAH; dynein axonemal heavy chain
K10409  DNAI1; dynein axonemal intermediate chain 1
K10410  DNALI; dynein axonemal light intermediate chain 1
K10411  DNAL1; dynein axonemal light chain 1
K10412  DNAL4; dynein axonemal light chain 4
K10424  DCTN2; dynactin 2
K10425  DCTN3; dynactin 3
K10426  DCTN4; dynactin 4
K10427  DCTN5; dynactin 5
K10428  DCTN6; dynactin 6
K10881  SHFM1, DSS1, RPN15; 26 proteasome complex subunit DSS1
K11143  DNAI2; dynein axonemal intermediate chain 2
K11351  NDUFB11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11
K11352  NDUFA12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 12
K11353  NDUFA13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13
K11644  SIN3A; paired amphipathic helix protein Sin3a
K11824  AP2A; AP-2 complex subunit alpha
K11825  AP2B1; AP-2 complex subunit beta-1
K11826  AP2M1; AP-2 complex subunit mu-1
K11827  AP2S1; AP-2 complex subunit sigma-1
K11828  DLG4, PSD95; discs large protein 4
K11829  RCOR1, COREST; REST corepressor 1
K11830  TFAM, MTTFA; transcription factor A, mitochondrial
K11831  NRF1; nuclear respiratory factor 1
K15040  VDAC2; voltage-dependent anion channel protein 2
K15041  VDAC3; voltage-dependent anion channel protein 3
K16575  ACTR1, ARP1; centractin
K16576  ACTR10, ARP11; actin-related protein 10
K17589  RB1CC1; RB1-inducible coiled-coil protein 1
K17889  ATG14L, ATG14; beclin 1-associated autophagy-related key regulator
K17906  ATG2; autophagy-related protein 2
K17908  WIPI1_2, ATG18; autophagy-related protein 18
K17985  AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
K19730  ATG101; autophagy-related protein 101
K21246  NRBF2; nuclear receptor-binding factor 2
K21357  ULK1; serine/threonine-protein kinase ULK1 [EC:2.7.11.1]
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
ko00190  Oxidative phosphorylation
ko03020  RNA polymerase
ko03022  Basal transcription factors
ko03050  Proteasome
ko04020  Calcium signaling pathway
ko04115  p53 signaling pathway
ko04140  Autophagy - animal
ko04141  Protein processing in endoplasmic reticulum
ko04144  Endocytosis
ko04210  Apoptosis
ko04724  Glutamatergic synapse

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