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PDBsum entry 1t3s

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protein metals links
Transport protein PDB id
1t3s
Jmol
Contents
Protein chain
294 a.a. *
Metals
_HG
Waters ×87
* Residue conservation analysis
PDB id:
1t3s
Name: Transport protein
Title: Structural analysis of the voltage-dependent calcium channel subunit functional core
Structure: Dihydropyridine-sensitive l-type, calcium channel subunit. Chain: a. Fragment: functional core (residues 25-422). Synonym: voltage-dependent calcium channel beta-2 subunit, engineered: yes. Mutation: yes
Source: Oryctolagus cuniculus. Rabbit. Organism_taxid: 9986. Gene: cacnb2, cacnlb2. Expressed in: escherichia coli bl21(de3). Expression_system_taxid: 469008.
Resolution:
2.30Å     R-factor:   0.262     R-free:   0.277
Authors: Y.Opatowsky,C.-C.Chen,K.P.Campbell,J.A.Hirsch
Key ref: Y.S.Choo et al. (2004). Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet, 13, 1407-1420. PubMed id: 15163634
Date:
27-Apr-04     Release date:   25-May-04    
PROCHECK
Go to PROCHECK summary
 Headers
 References

Protein chain
Pfam   ArchSchema ?
P54288  (CACB2_RABIT) -  Voltage-dependent L-type calcium channel subunit beta-2
Seq:
Struc:
 
Seq:
Struc:
632 a.a.
294 a.a.*
Key:    PfamA domain  Secondary structure  CATH domain
* PDB and UniProt seqs differ at 4 residue positions (black crosses)

 Gene Ontology (GO) functional annotation 
  GO annot!
  Cellular component     voltage-gated calcium channel complex   1 term 
  Biological process     calcium ion transmembrane transport   1 term 
  Biochemical function     voltage-gated calcium channel activity     1 term  

 

 
Hum Mol Genet 13:1407-1420 (2004)
PubMed id: 15163634  
 
 
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release.
Y.S.Choo, G.V.Johnson, M.MacDonald, P.J.Detloff, M.Lesort.
 
  ABSTRACT  
 
Huntington's disease (HD) is initiated by an abnormally expanded polyglutamine stretch in the huntingtin protein, conferring a novel property on the protein that leads to the loss of striatal neurons. Defects in mitochondrial function have been implicated in the pathogenesis of HD. Here, we have examined the hypothesis that the mutant huntingtin protein may directly interact with the mitochondrion and affect its function. In human neuroblastoma cells and clonal striatal cells established from HdhQ7 (wild-type) and HdhQ111 (mutant) homozygote mouse knock-in embryos, huntingtin was present in a purified mitochondrial fraction. Subfractionation of the mitochondria and limited trypsin digestion of the organelle demonstrated that huntingtin was associated with the outer mitochondrial membrane. We further demonstrated that a recombinant truncated mutant huntingtin protein, but not a wild-type, directly induced mitochondrial permeability transition (MPT) pore opening in isolated mouse liver mitochondria, an effect that was prevented completely by cyclosporin A (CSA) and ATP. Importantly, the mutant huntingtin protein significantly decreased the Ca2+ threshold necessary to trigger MPT pore opening. We found a similar increased susceptibility to the calcium-induced MPT in liver mitochondria isolated from a knock-in HD mouse model. The mutant huntingtin protein-induced MPT pore opening was accompanied by a significant release of cytochrome c, an effect completely inhibited by CSA. These findings suggest that the development of specific MPT inhibitors may be an interesting therapeutic avenue to delay the onset of HD.
 

Literature references that cite this PDB file's key reference

  PubMed id Reference
21153060 F.M.Ribeiro, R.G.Pires, and S.S.Ferguson (2011).
Huntington's disease and Group I metabotropic glutamate receptors.
  Mol Neurobiol, 43, 1.  
21285522 F.Mochel, and R.G.Haller (2011).
Energy deficit in Huntington disease: why it matters.
  J Clin Invest, 121, 493-499.  
20730552 S.Ramakrishna, B.Suresh, and K.H.Baek (2011).
The role of deubiquitinating enzymes in apoptosis.
  Cell Mol Life Sci, 68, 15-26.  
21257639 U.Shirendeb, A.P.Reddy, M.Manczak, M.J.Calkins, P.Mao, D.A.Tagle, and P.Hemachandra Reddy (2011).
Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage.
  Hum Mol Genet, 20, 1438-1455.  
21336284 W.Song, J.Chen, A.Petrilli, G.Liot, E.Klinglmayr, Y.Zhou, P.Poquiz, J.Tjong, M.A.Pouladi, M.R.Hayden, E.Masliah, M.Ellisman, I.Rouiller, R.Schwarzenbacher, B.Bossy, G.Perkins, and E.Bossy-Wetzel (2011).
Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity.
  Nat Med, 17, 377-382.  
19642201 A.Valencia, P.B.Reeves, E.Sapp, X.Li, J.Alexander, K.B.Kegel, K.Chase, N.Aronin, and M.DiFiglia (2010).
Mutant huntingtin and glycogen synthase kinase 3-beta accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease.
  J Neurosci Res, 88, 179-190.  
20653508 C.Rodolfo, F.Ciccosanti, G.D.Giacomo, M.Piacentini, and G.M.Fimia (2010).
Proteomic analysis of mitochondrial dysfunction in neurodegenerative diseases.
  Expert Rev Proteomics, 7, 519-542.  
20480217 C.Turner, and A.H.Schapira (2010).
Mitochondrial matters of the brain: the role in Huntington's disease.
  J Bioenerg Biomembr, 42, 193-198.  
20558522 G.M.Perry, S.Tallaksen-Greene, A.Kumar, M.Y.Heng, A.Kneynsberg, T.van Groen, P.J.Detloff, R.L.Albin, and M.Lesort (2010).
Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease.
  Hum Mol Genet, 19, 3354-3371.  
  20398440 I.P.de Castro, L.M.Martins, and R.Tufi (2010).
Mitochondrial quality control and neurological disease: an emerging connection.
  Expert Rev Mol Med, 12, e12.  
20454921 J.M.Oliveira (2010).
Mitochondrial bioenergetics and dynamics in Huntington's disease: tripartite synapses and selective striatal degeneration.
  J Bioenerg Biomembr, 42, 227-234.  
20464463 M.Pandey, K.P.Mohanakumar, and R.Usha (2010).
Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease.
  J Bioenerg Biomembr, 42, 217-226.  
20529956 R.K.Chaturvedi, N.Y.Calingasan, L.Yang, T.Hennessey, A.Johri, and M.F.Beal (2010).
Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation.
  Hum Mol Genet, 19, 3190-3205.  
  21069748 V.Costa, M.Giacomello, R.Hudec, R.Lopreiato, G.Ermak, D.Lim, W.Malorni, K.J.Davies, E.Carafoli, and L.Scorrano (2010).
Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli.
  EMBO Mol Med, 2, 490-503.  
20524050 V.Pérez-De la Cruz, P.Carrillo-Mora, and A.Santamaría (2010).
Huntington's disease and mitochondrial alterations: emphasis on experimental models.
  J Bioenerg Biomembr, 42, 207-215.  
19591925 X.J.Li, A.L.Orr, and S.Li (2010).
Impaired mitochondrial trafficking in Huntington's disease.
  Biochim Biophys Acta, 1802, 62-65.  
20556492 Y.N.Jin, and G.V.Johnson (2010).
The interrelationship between mitochondrial dysfunction and transcriptional dysregulation in Huntington disease.
  J Bioenerg Biomembr, 42, 199-205.  
19564950 E.Seppet, M.Gruno, A.Peetsalu, Z.Gizatullina, H.P.Nguyen, S.Vielhaber, M.H.Wussling, S.Trumbeckaite, O.Arandarcikaite, D.Jerzembeck, M.Sonnabend, K.Jegorov, S.Zierz, F.Striggow, and F.N.Gellerich (2009).
Mitochondria and energetic depression in cell pathophysiology.
  Int J Mol Sci, 10, 2252-2303.  
19268425 F.Celsi, P.Pizzo, M.Brini, S.Leo, C.Fotino, P.Pinton, and R.Rizzuto (2009).
Mitochondria, calcium and cell death: a deadly triad in neurodegeneration.
  Biochim Biophys Acta, 1787, 335-344.  
19300456 G.Liot, B.Bossy, S.Lubitz, Y.Kushnareva, N.Sejbuk, and E.Bossy-Wetzel (2009).
Complex II inhibition by 3-NP causes mitochondrial fragmentation and neuronal cell death via an NMDA- and ROS-dependent pathway.
  Cell Death Differ, 16, 899-909.  
19825937 G.Z.Tao, K.S.Looi, D.M.Toivola, P.Strnad, Q.Zhou, J.Liao, Y.Wei, A.Habtezion, and M.B.Omary (2009).
Keratins modulate the shape and function of hepatocyte mitochondria: a mechanism for protection from apoptosis.
  J Cell Sci, 122, 3851-3855.  
19447173 H.Liang, Q.Ran, Y.C.Jang, D.Holstein, J.Lechleiter, T.McDonald-Marsh, A.Musatov, W.Song, H.Van Remmen, and A.Richardson (2009).
Glutathione peroxidase 4 differentially regulates the release of apoptogenic proteins from mitochondria.
  Free Radic Biol Med, 47, 312-320.  
19229105 H.Xiong, D.Wang, L.Chen, Y.S.Choo, H.Ma, C.Tang, K.Xia, W.Jiang, Z.Ronai, X.Zhuang, and Z.Zhang (2009).
Parkin, PINK1, and DJ-1 form a ubiquitin E3 ligase complex promoting unfolded protein degradation.
  J Clin Invest, 119, 650-660.  
19185022 J.Wu, H.K.Jeong, S.E.Bulin, S.W.Kwon, J.H.Park, and I.Bezprozvanny (2009).
Ginsenosides protect striatal neurons in a cellular model of Huntington's disease.
  J Neurosci Res, 87, 1904-1912.  
19566678 K.B.Kegel, E.Sapp, J.Alexander, A.Valencia, P.Reeves, X.Li, N.Masso, L.Sobin, N.Aronin, and M.DiFiglia (2009).
Polyglutamine expansion in huntingtin alters its interaction with phospholipids.
  J Neurochem, 110, 1585-1597.  
19460457 M.S.Parihar, A.Parihar, M.Fujita, M.Hashimoto, and P.Ghafourifar (2009).
Alpha-synuclein overexpression and aggregation exacerbates impairment of mitochondrial functions by augmenting oxidative stress in human neuroblastoma cells.
  Int J Biochem Cell Biol, 41, 2015-2024.  
19250966 P.G.Mastroberardino, E.K.Hoffman, M.P.Horowitz, R.Betarbet, G.Taylor, D.Cheng, H.M.Na, C.A.Gutekunst, M.Gearing, J.Q.Trojanowski, M.Anderson, C.T.Chu, J.Peng, and J.T.Greenamyre (2009).
A novel transferrin/TfR2-mediated mitochondrial iron transport system is disrupted in Parkinson's disease.
  Neurobiol Dis, 34, 417-431.  
19394359 P.H.Reddy, P.Mao, and M.Manczak (2009).
Mitochondrial structural and functional dynamics in Huntington's disease.
  Brain Res Rev, 61, 33-48.  
19419557 P.Marambaud, U.Dreses-Werringloer, and V.Vingtdeux (2009).
Calcium signaling in neurodegeneration.
  Mol Neurodegener, 4, 20.  
19650870 P.O.Bauer, and N.Nukina (2009).
The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies.
  J Neurochem, 110, 1737-1765.  
19622387 R.A.Quintanilla, and G.V.Johnson (2009).
Role of mitochondrial dysfunction in the pathogenesis of Huntington's disease.
  Brain Res Bull, 80, 242-247.  
19460884 R.K.Chaturvedi, P.Adhihetty, S.Shukla, T.Hennessy, N.Calingasan, L.Yang, A.Starkov, M.Kiaei, M.Cannella, J.Sassone, A.Ciammola, F.Squitieri, and M.F.Beal (2009).
Impaired PGC-1alpha function in muscle in Huntington's disease.
  Hum Mol Genet, 18, 3048-3065.  
19498170 S.Subramaniam, K.M.Sixt, R.Barrow, and S.H.Snyder (2009).
Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity.
  Science, 324, 1327-1330.  
18337408 A.L.Orr, S.Li, C.E.Wang, H.Li, J.Wang, J.Rong, X.Xu, P.G.Mastroberardino, J.T.Greenamyre, and X.J.Li (2008).
N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking.
  J Neurosci, 28, 2783-2792.  
18418676 B.R.Underwood, and D.C.Rubinsztein (2008).
Spinocerebellar ataxias caused by polyglutamine expansions: A review of therapeutic strategies.
  Cerebellum, 7, 215-221.  
18951640 E.Bossy-Wetzel, A.Petrilli, and A.B.Knott (2008).
Mutant huntingtin and mitochondrial dysfunction.
  Trends Neurosci, 31, 609-616.  
18606820 F.N.Gellerich, Z.Gizatullina, H.P.Nguyen, S.Trumbeckaite, S.Vielhaber, E.Seppet, S.Zierz, B.Landwehrmeyer, O.Riess, S.von Hörsten, and F.Striggow (2008).
Impaired regulation of brain mitochondria by extramitochondrial Ca2+ in transgenic Huntington disease rats.
  J Biol Chem, 283, 30715-30724.  
18445124 G.W.Araújo, C.Beyer, and S.Arnold (2008).
Oestrogen influences on mitochondrial gene expression and respiratory chain activity in cortical and mesencephalic astrocytes.
  J Neuroendocrinol, 20, 930-941.  
18502655 H.Zhang, Q.Li, R.K.Graham, E.Slow, M.R.Hayden, and I.Bezprozvanny (2008).
Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease.
  Neurobiol Dis, 31, 80-88.  
18412970 H.Zhang, S.Das, Q.Z.Li, I.Dragatsis, J.Repa, S.Zeitlin, G.Hajnóczky, and I.Bezprozvanny (2008).
Elucidating a normal function of huntingtin by functional and microarray analysis of huntingtin-null mouse embryonic fibroblasts.
  BMC Neurosci, 9, 38.  
18588526 J.M.Gil, and A.C.Rego (2008).
Mechanisms of neurodegeneration in Huntington's disease.
  Eur J Neurosci, 27, 2803-2820.  
18214990 K.Toriumi, Y.Oma, Y.Kino, E.Futai, N.Sasagawa, and S.Ishiura (2008).
Expression of polyalanine stretches induces mitochondrial dysfunction.
  J Neurosci Res, 86, 1529-1537.  
19081372 M.P.Mattson, M.Gleichmann, and A.Cheng (2008).
Mitochondria in neuroplasticity and neurological disorders.
  Neuron, 60, 748-766.  
19076459 R.K.Chaturvedi, and M.F.Beal (2008).
Mitochondrial approaches for neuroprotection.
  Ann N Y Acad Sci, 1147, 395-412.  
18333761 S.DiMauro, and E.A.Schon (2008).
Mitochondrial disorders in the nervous system.
  Annu Rev Neurosci, 31, 91.  
17976163 S.T.Lee, K.Chu, J.E.Park, N.H.Hong, W.S.Im, L.Kang, Z.Han, K.H.Jung, M.W.Kim, and M.Kim (2008).
Atorvastatin attenuates mitochondrial toxin-induced striatal degeneration, with decreasing iNOS/c-Jun levels and activating ERK/Akt pathways.
  J Neurochem, 104, 1190-1200.  
18221365 X.Xifró, J.M.García-Martínez, D.Del Toro, J.Alberch, and E.Pérez-Navarro (2008).
Calcineurin is involved in the early activation of NMDA-mediated cell death in mutant huntingtin knock-in striatal cells.
  J Neurochem, 105, 1596-1612.  
17708681 J.M.Lee, E.V.Ivanova, I.S.Seong, T.Cashorali, I.Kohane, J.F.Gusella, and M.E.MacDonald (2007).
Unbiased gene expression analysis implicates the huntingtin polyglutamine tract in extra-mitochondrial energy metabolism.
  PLoS Genet, 3, e135.  
17394466 J.M.Oliveira, M.B.Jekabsons, S.Chen, A.Lin, A.C.Rego, J.Gonçalves, L.M.Ellerby, and D.G.Nicholls (2007).
Mitochondrial dysfunction in Huntington's disease: the bioenergetics of isolated and in situ mitochondria from transgenic mice.
  J Neurochem, 101, 241-249.  
16860906 J.Paz, H.Yao, H.S.Lim, X.Y.Lu, and W.Zhang (2007).
The neuroprotective role of attractin in neurodegeneration.
  Neurobiol Aging, 28, 1446-1456.  
17126554 M.J.Mihm, D.M.Amann, B.L.Schanbacher, R.A.Altschuld, J.A.Bauer, and K.R.Hoyt (2007).
Cardiac dysfunction in the R6/2 mouse model of Huntington's disease.
  Neurobiol Dis, 25, 297-308.  
17663643 R.H.Swerdlow (2007).
Treating neurodegeneration by modifying mitochondria: potential solutions to a "complex" problem.
  Antioxid Redox Signal, 9, 1591-1603.  
18000542 T.Ghosh, N.Pandey, A.Maitra, S.K.Brahmachari, and B.Pillai (2007).
A role for voltage-dependent anion channel Vdac1 in polyglutamine-mediated neuronal cell death.
  PLoS ONE, 2, e1170.  
17435251 W.Liu, R.Gnanasambandam, J.Benjamin, G.Kaur, P.B.Getman, A.J.Siegel, R.D.Shortridge, and S.Singh (2007).
Mutations in cytochrome c oxidase subunit VIa cause neurodegeneration and motor dysfunction in Drosophila.
  Genetics, 176, 937-946.  
16452635 A.Benchoua, Y.Trioulier, D.Zala, M.C.Gaillard, N.Lefort, N.Dufour, F.Saudou, J.M.Elalouf, E.Hirsch, P.Hantraye, N.Déglon, and E.Brouillet (2006).
Involvement of mitochondrial complex II defects in neuronal death produced by N-terminus fragment of mutated huntingtin.
  Mol Biol Cell, 17, 1652-1663.  
16983659 E.A.Thomas (2006).
Striatal specificity of gene expression dysregulation in Huntington's disease.
  J Neurosci Res, 84, 1151-1164.  
17081970 J.K.McGill, and M.F.Beal (2006).
PGC-1alpha, a new therapeutic target in Huntington's disease?
  Cell, 127, 465-468.  
16805775 J.Q.Kwong, M.F.Beal, and G.Manfredi (2006).
The role of mitochondria in inherited neurodegenerative diseases.
  J Neurochem, 97, 1659-1675.  
16606366 K.Furukawa, M.Matsuzaki-Kobayashi, T.Hasegawa, A.Kikuchi, N.Sugeno, Y.Itoyama, Y.Wang, P.J.Yao, I.Bushlin, and A.Takeda (2006).
Plasma membrane ion permeability induced by mutant alpha-synuclein contributes to the degeneration of neural cells.
  J Neurochem, 97, 1071-1077.  
17051205 M.T.Lin, and M.F.Beal (2006).
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.
  Nature, 443, 787-795.  
17034350 S.E.Browne, and M.F.Beal (2006).
Oxidative damage in Huntington's disease pathogenesis.
  Antioxid Redox Signal, 8, 2061-2073.  
16623826 Z.Mao, Y.S.Choo, and M.Lesort (2006).
Cystamine and cysteamine prevent 3-NP-induced mitochondrial depolarization of Huntington's disease knock-in striatal cells.
  Eur J Neurosci, 23, 1701-1710.  
16437579 Z.Z.Gizatullina, K.S.Lindenberg, P.Harjes, Y.Chen, C.M.Kosinski, B.G.Landwehrmeyer, A.C.Ludolph, F.Striggow, S.Zierz, and F.N.Gellerich (2006).
Low stability of Huntington muscle mitochondria against Ca2+ in R6/2 mice.
  Ann Neurol, 59, 407-411.  
16316967 A.Maloyan, A.Sanbe, H.Osinska, M.Westfall, D.Robinson, K.Imahashi, E.Murphy, and J.Robbins (2005).
Mitochondrial dysfunction and apoptosis underlie the pathogenic process in alpha-B-crystallin desmin-related cardiomyopathy.
  Circulation, 112, 3451-3461.  
15967379 D.Goffredo, D.Rigamonti, C.Zuccato, M.Tartari, M.Valenza, and E.Cattaneo (2005).
Prevention of cytosolic IAPs degradation: a potential pharmacological target in Huntington's Disease.
  Pharmacol Res, 52, 140-150.  
16300642 E.Brouillet, C.Jacquard, N.Bizat, and D.Blum (2005).
3-Nitropropionic acid: a mitochondrial toxin to uncover physiopathological mechanisms underlying striatal degeneration in Huntington's disease.
  J Neurochem, 95, 1521-1540.  
16115016 G.D.Zeevalk, L.P.Bernard, C.Song, M.Gluck, and J.Ehrhart (2005).
Mitochondrial inhibition and oxidative stress: reciprocating players in neurodegeneration.
  Antioxid Redox Signal, 7, 1117-1139.  
15721979 I.G.Stavrovskaya, and B.S.Kristal (2005).
The powerhouse takes control of the cell: is the mitochondrial permeability transition a viable therapeutic target against neuronal dysfunction and death?
  Free Radic Biol Med, 38, 687-697.  
16178023 M.F.Beal (2005).
Mitochondria take center stage in aging and neurodegeneration.
  Ann Neurol, 58, 495-505.  
15935052 N.Brustovetsky, R.LaFrance, K.J.Purl, T.Brustovetsky, C.D.Keene, W.C.Low, and J.M.Dubinsky (2005).
Age-dependent changes in the calcium sensitivity of striatal mitochondria in mouse models of Huntington's Disease.
  J Neurochem, 93, 1361-1370.  
15924570 R.LaFrance, N.Brustovetsky, C.Sherburne, D.Delong, and J.M.Dubinsky (2005).
Age-related changes in regional brain mitochondria from Fischer 344 rats.
  Aging Cell, 4, 139-145.  
16104847 S.Marubuchi, Y.Wada, T.Okuda, Y.Hara, M.L.Qi, M.Hoshino, M.Nakagawa, I.Kanazawa, and H.Okazawa (2005).
Polyglutamine tract-binding protein-1 dysfunction induces cell death of neurons through mitochondrial stress.
  J Neurochem, 95, 858-870.  
16341586 S.Y.Kim, L.Marekov, P.Bubber, S.E.Browne, I.Stavrovskaya, J.Lee, P.M.Steinert, J.P.Blass, M.F.Beal, G.E.Gibson, and A.J.Cooper (2005).
Mitochondrial aconitase is a transglutaminase 2 substrate: transglutamination is a probable mechanism contributing to high-molecular-weight aggregates of aconitase and loss of aconitase activity in Huntington disease brain.
  Neurochem Res, 30, 1245-1255.  
16087879 T.Nguyen, A.Hamby, and S.M.Massa (2005).
Clioquinol down-regulates mutant huntingtin expression in vitro and mitigates pathology in a Huntington's disease mouse model.
  Proc Natl Acad Sci U S A, 102, 11840-11845.  
15695335 T.S.Tang, E.Slow, V.Lupu, I.G.Stavrovskaya, M.Sugimori, R.Llinás, B.S.Kristal, M.R.Hayden, and I.Bezprozvanny (2005).
Disturbed Ca2+ signaling and apoptosis of medium spiny neurons in Huntington's disease.
  Proc Natl Acad Sci U S A, 102, 2602-2607.  
15598740 D.W.Colby, Y.Chu, J.P.Cassady, M.Duennwald, H.Zazulak, J.M.Webster, A.Messer, S.Lindquist, V.M.Ingram, and K.D.Wittrup (2004).
Potent inhibition of huntingtin aggregation and cytotoxicity by a disulfide bond-free single-domain intracellular antibody.
  Proc Natl Acad Sci U S A, 101, 17616-17621.  
15548647 R.J.Ferrante, H.Ryu, J.K.Kubilus, S.D'Mello, K.L.Sugars, J.Lee, P.Lu, K.Smith, S.Browne, M.F.Beal, B.S.Kristal, I.G.Stavrovskaya, S.Hewett, D.C.Rubinsztein, B.Langley, and R.R.Ratan (2004).
Chemotherapy for the brain: the antitumor antibiotic mithramycin prolongs survival in a mouse model of Huntington's disease.
  J Neurosci, 24, 10335-10342.  
The most recent references are shown first. Citation data come partly from CiteXplore and partly from an automated harvesting procedure. Note that this is likely to be only a partial list as not all journals are covered by either method. However, we are continually building up the citation data so more and more references will be included with time.