Gillian P Bates

Summary

Affiliation: King's College London
Country: UK

Publications

  1. ncbi request reprint History of genetic disease: the molecular genetics of Huntington disease - a history
    Gillian P Bates
    Department of Medical and Molecular Genetics, GKT School of Medicine, King s College London, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, United Kingdom
    Nat Rev Genet 6:766-73. 2005
  2. ncbi request reprint BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By
    Gillian P Bates
    King s College London School of Medicine, London SE1 9RT, UK
    Science 311:1385-6. 2006
  3. ncbi request reprint The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes
    Ben Woodman
    Department of Medical and Molecular Genetics, King s College London School of Medicine, London, UK
    Brain Res Bull 72:83-97. 2007
  4. pmc HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration
    Michal Mielcarek
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS Biol 11:e1001717. 2013
  5. pmc Suppression of protein aggregation by chaperone modification of high molecular weight complexes
    John Labbadia
    Department of Medical and Molecular Genetics, King s College London, London SE1 9RT, UK
    Brain 135:1180-96. 2012
  6. ncbi request reprint Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease
    Caroline L Benn
    King s College London, Medical and Molecular Genetics, GKT School of Medicine, UK
    Hum Mol Genet 14:3065-78. 2005
  7. ncbi request reprint Minocycline and doxycycline are not beneficial in a model of Huntington's disease
    Donna L Smith
    Department of Medical and Molecular Genetics, King s College London, United Kingdom
    Ann Neurol 54:186-96. 2003
  8. pmc Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease
    Christian Landles
    Department Medical and Molecular Genetics, King s College London School of Medicine, King s College London, London SE1 9RT, United Kingdom
    J Biol Chem 285:8808-23. 2010
  9. pmc Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease
    Caroline L Benn
    Department of Medical and Molecular Genetics, King s College London School of Medicine, King s College London, London, UK
    PLoS ONE 4:e5747. 2009
  10. pmc The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease
    John S Bett
    King s College London School of Medicine, Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    J Cell Mol Med 13:2645-57. 2009

Detail Information

Publications56

  1. ncbi request reprint History of genetic disease: the molecular genetics of Huntington disease - a history
    Gillian P Bates
    Department of Medical and Molecular Genetics, GKT School of Medicine, King s College London, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, United Kingdom
    Nat Rev Genet 6:766-73. 2005
    ..We are now equipped with powerful genetic models that continue to uncover new aspects of the pathogenesis of Huntington disease and will be instrumental for the development of therapeutic approaches for this disease...
  2. ncbi request reprint BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By
    Gillian P Bates
    King s College London School of Medicine, London SE1 9RT, UK
    Science 311:1385-6. 2006
  3. ncbi request reprint The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes
    Ben Woodman
    Department of Medical and Molecular Genetics, King s College London School of Medicine, London, UK
    Brain Res Bull 72:83-97. 2007
    ..This supports the continued use of the more high-throughput fragment models to identify mechanisms of pathogenesis and for preclinical screening...
  4. pmc HDAC4 reduction: a novel therapeutic strategy to target cytoplasmic huntingtin and ameliorate neurodegeneration
    Michal Mielcarek
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS Biol 11:e1001717. 2013
    ..HDAC4 reduction presents a novel strategy for targeting huntingtin aggregation, which may be amenable to small-molecule therapeutics. ..
  5. pmc Suppression of protein aggregation by chaperone modification of high molecular weight complexes
    John Labbadia
    Department of Medical and Molecular Genetics, King s College London, London SE1 9RT, UK
    Brain 135:1180-96. 2012
    ..Furthermore, our findings represent an important proof of principle that DNAJ manipulation is a valid therapeutic approach for intervention in Huntington's disease...
  6. ncbi request reprint Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington's disease
    Caroline L Benn
    King s College London, Medical and Molecular Genetics, GKT School of Medicine, UK
    Hum Mol Genet 14:3065-78. 2005
    ..However, our data suggest that cytoplasmic mutant exon 1 htt, if present, contributes to disease progression...
  7. ncbi request reprint Minocycline and doxycycline are not beneficial in a model of Huntington's disease
    Donna L Smith
    Department of Medical and Molecular Genetics, King s College London, United Kingdom
    Ann Neurol 54:186-96. 2003
    ..In the light of these new data, we would advise that caution be exercised in proceeding into human clinical trials of minocycline...
  8. pmc Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington disease
    Christian Landles
    Department Medical and Molecular Genetics, King s College London School of Medicine, King s College London, London SE1 9RT, United Kingdom
    J Biol Chem 285:8808-23. 2010
    ..This methodology can be used to validate the inhibition of specific proteases as therapeutic targets for HD by pharmacological or genetic approaches...
  9. pmc Genetic knock-down of HDAC7 does not ameliorate disease pathogenesis in the R6/2 mouse model of Huntington's disease
    Caroline L Benn
    Department of Medical and Molecular Genetics, King s College London School of Medicine, King s College London, London, UK
    PLoS ONE 4:e5747. 2009
    ..Therefore, we conclude that the beneficial effects of HDAC inhibitors are not predominantly mediated through the inhibition of HDAC7...
  10. pmc The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease
    John S Bett
    King s College London School of Medicine, Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    J Cell Mol Med 13:2645-57. 2009
    ..These results suggest that the expression of aggregation-prone mutant htt causes disturbances to the ubiquitin system, which may contribute to disease due to the diverse and important roles of ubiquitin...
  11. pmc Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease
    Hilary Moffitt
    Department of Medical and Molecular Genetics, King s College London School of Medicine, London, United Kingdom
    PLoS ONE 4:e8025. 2009
    ....
  12. ncbi request reprint Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation
    Alexandra Zourlidou
    Department of Medical and Molecular Genetics, King s College London, School of Medicine, London SE1 9RT, UK
    Hum Mol Genet 16:1078-90. 2007
    ..Our study is the first to suggest a differential modulation of Hsp27 activation in vivo and, importantly, it illustrates the diverse effect of Hsp27 on acute versus chronic models of disease...
  13. ncbi request reprint Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington's disease
    Emma Hockly
    King s College London, Department of Medical and Molecular Genetics, GKT School of Medicine, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, UK
    Neurobiol Dis 21:228-36. 2006
    ..At the maximum tolerated dose, we achieved steady-state riluzole levels of 100 microM in brain. However, this was insufficient to inhibit aggregation in vivo and we found no improvement in the disease phenotype...
  14. pmc Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease
    Kirupa Sathasivam
    Department of Medical and Molecular Genetics, King s College London, London SE1 9RT, United Kingdom
    Proc Natl Acad Sci U S A 110:2366-70. 2013
    ..RNA-targeted therapeutic strategies designed to lower the levels of HTT are under development. Many of these approaches would not prevent the production of exon 1 HTT and should be reviewed in light of our findings...
  15. pmc SAHA decreases HDAC 2 and 4 levels in vivo and improves molecular phenotypes in the R6/2 mouse model of Huntington's disease
    Michal Mielcarek
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS ONE 6:e27746. 2011
    ..We found that SAHA reduces SDS-insoluble aggregate load in the cortex and brain stem but not in the hippocampus of the R6/2 brains, and that this was accompanied by restoration of Bdnf cortical transcript levels...
  16. pmc Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease
    Kirupa Sathasivam
    Department of Medical and Molecular Genetics, King s College London School of Medicine, King s College London, 8th Floor Tower Wing, Guy s Tower, Great Maze Pond, London, UK
    Hum Mol Genet 19:65-78. 2010
    ....
  17. ncbi request reprint Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target
    John S Bett
    Department of Medical and Molecular Genetics, GKT School of Medicine, King s College London, UK
    Hum Mol Genet 15:33-44. 2006
    ..These findings suggest that REGgamma is not a viable therapeutic target in polyglutamine disease and that overall proteasome function is not impaired by trapped mutant polyglutamine in R6/2 mice...
  18. pmc The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease
    John S Bett
    Department of Medical and Molecular Genetics, King s College London School of Medicine, King s College London, London, United Kingdom
    PLoS ONE 4:e5128. 2009
    ..It is therefore likely that the relationship between mutant polyglutamine proteins and the UPS is more complex than originally anticipated...
  19. pmc Hdac6 knock-out increases tubulin acetylation but does not modify disease progression in the R6/2 mouse model of Huntington's disease
    Anna Bobrowska
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS ONE 6:e20696. 2011
    ..Therefore, we conclude that HDAC6 inhibition does not modify disease progression in R6/2 mice and HDAC6 should not be prioritized as a therapeutic target for HD...
  20. pmc DNA instability in postmitotic neurons
    Roman Gonitel
    Department of Medical and Molecular Genetics, King s College London School of Medicine, London SE1 9RT, United Kingdom
    Proc Natl Acad Sci U S A 105:3467-72. 2008
    ....
  21. pmc Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/2 mouse model of HD
    Ivan Rattray
    King s College London, Institute of Psychiatry, Department of Neuroscience, London, United Kingdom
    PLoS ONE 8:e60012. 2013
    ..In conclusion, modeling pre-manifest and early progression of the disease in more slowly progressing animal models will be key to establishing which changes are causally related...
  22. pmc Altered chromatin architecture underlies progressive impairment of the heat shock response in mouse models of Huntington disease
    John Labbadia
    Department of Medical and Molecular Genetics, King s College London, London, UK
    J Clin Invest 121:3306-19. 2011
    ..Consequently, pharmacological induction of HSF1 as a therapeutic approach to HD is more complex than was previously anticipated...
  23. ncbi request reprint Progressive decrease in chaperone protein levels in a mouse model of Huntington's disease and induction of stress proteins as a therapeutic approach
    David G Hay
    Medical and Molecular Genetics, GKT School of Medicine, King s College, London, UK
    Hum Mol Genet 13:1389-405. 2004
    ..Radicicol and geldanamycin could both maintain chaperone induction for at least 3 weeks and alter the detergent soluble properties of polyQ aggregates over this time course...
  24. ncbi request reprint Abnormal phosphorylation of synapsin I predicts a neuronal transmission impairment in the R6/2 Huntington's disease transgenic mice
    Jean Charles Liévens
    Medical and Molecular Genetics, GKT School of Medicine, London SE1 9RT, United Kingdom
    Mol Cell Neurosci 20:638-48. 2002
    ..Together the results suggest that an early impairment in synapsin phosphorylation-dephosphorylation may alter synaptic vesicle trafficking and lead to defective neurotransmission in HD...
  25. ncbi request reprint Monitoring aggregate formation in organotypic slice cultures from transgenic mice
    Donna L Smith
    Department of Medical and Molecular Genetics, GKT School of Medicine, King s College, Guy s Hospital, London, UK
    Methods Mol Biol 277:161-71. 2004
    ..This information is essential to the planning and application of an in vivo drug trial in the R6/2 mice...
  26. ncbi request reprint Experimental therapeutics in Huntington's disease: are models useful for therapeutic trials?
    Gillian P Bates
    King s College London, Guy s Hospital, London SE1 9RT, UK
    Curr Opin Neurol 16:465-70. 2003
    ..The role that disease models will play in this process is discussed...
  27. pmc Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
    Emma Hockly
    Medical and Molecular Genetics, Guy s, King s and St Thomas School of Medicine, King s College London, Eighth Floor Guy s Tower, Guy s Hospital, London SE1 9RT, United Kingdom
    Proc Natl Acad Sci U S A 100:2041-6. 2003
    ..SAHA dramatically improved the motor impairment in R6/2 mice, clearly validating the pursuit of this class of compounds as HD therapeutics...
  28. pmc Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series
    Christian Landles
    Neurogenetics Laboratory, Medical and Molecular Genetics, GKT School of Medicine, King s College London, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, UK
    EMBO Rep 5:958-63. 2004
    ..We then focus on evidence that supports a role for transcriptional dysfunction and impaired protein folding and degradation as early events in disease pathogenesis...
  29. ncbi request reprint Histone deacetylase inhibitors as therapeutics for polyglutamine disorders
    Rachel Butler
    King s College London School of Medicine, Department of Medical and Molecular Genetics, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, UK
    Nat Rev Neurosci 7:784-96. 2006
    ..Here, we discuss the potential therapeutic pathways through which histone deacetylase inhibitors might act to correct the aberrant transcription observed in Huntington's disease and other polyglutamine repeat diseases...
  30. pmc HDAC4 does not act as a protein deacetylase in the postnatal murine brain in vivo
    Michal Mielcarek
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS ONE 8:e80849. 2013
    ..The lack of a major effect on the transcriptional profile is consistent with the cytoplasmic location of HDAC4 in the P3 murine brain. ..
  31. pmc Genetic knock-down of HDAC3 does not modify disease-related phenotypes in a mouse model of Huntington's disease
    Lara Moumné
    Department of Medical and Molecular Genetics, King s College London, London, United Kingdom
    PLoS ONE 7:e31080. 2012
    ..We conclude that HDAC3 should not be considered as the major mediator of the beneficial effect induced by SAHA and other HDAC inhibitors in HD...
  32. pmc Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease
    Ivan Rattray
    King s College London, Institute of Psychiatry, Department of Neuroscience, London, United Kingdom King s College London, Department of Medical and Molecular Genetics, London, United Kingdom
    PLoS ONE 8:e84726. 2013
    ..In conclusion, R6/1 mice exhibit many features of HD, but the underlying mechanisms driving these clear behavioral disturbances and the brain volume loss, still remain unclear. ..
  33. ncbi request reprint Mouse models of triplet repeat diseases
    Gillian P Bates
    King s College London, Department of Medical and Molecular Genetics, GKT School of Medicine, 8th Floor Guy s Tower, Guy s Hospital, London SE1 9RT, United Kingdom
    Mol Biotechnol 32:147-58. 2006
    ....
  34. ncbi request reprint Mouse models of triplet repeat diseases
    Gillian P Bates
    Department of Medical and Molecular Genetics, GKT School of Medicine, King s College, Guy s Hospital, London, UK
    Methods Mol Biol 277:3-15. 2004
    ..Mouse models have proved extremely useful in these goals and are now also being used for the preclinical testing of therapeutic compounds. This chapter reviews the successes and limitations of the approaches that have been developed...
  35. pmc Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders
    Alisia Carnemolla
    Department Medical and Molecular Genetics, King s College London, 8th Floor Tower Wing, Guy s Hosptial, Great Maze Pond, London SE1 9RT, UK
    Hum Mol Genet 23:3641-56. 2014
    ..This could result in a decrease in the protein folding capacity of old hearts with implications for age-related cardiac disorders. ..
  36. ncbi request reprint Novel Isoforms of Heat Shock Transcription Factor 1, HSF1γα and HSF1γβ, Regulate Chaperone Protein Gene Transcription
    Andreas Neueder
    King s College London, United Kingdom
    J Biol Chem . 2014
    ..Collectively, our observations suggest that the expression of HSF1 isoforms in a specific ratio provides an additional layer in the regulation of heat shock protein gene transcription...
  37. pmc Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease
    Caroline L Benn
    Medical and Molecular Genetics, King s College London School of Medicine, 8th Floor Tower Wing, Guy s Hospital, Great Maze Pond, London, SE1 9RT, UK
    Mol Neurodegener 3:17. 2008
    ..To remedy this situation, 12 housekeeping genes were examined to identify suitable reference genes for use in expression assays...
  38. ncbi request reprint Environmental enrichment slows disease progression in R6/2 Huntington's disease mice
    Emma Hockly
    Division of Medical and Molecular Genetics, GKT School of Medicine, King s College London, UK
    Ann Neurol 51:235-42. 2002
    ..Enrichment also delayed the loss of peristriatal cerebral volume in R6/2 brains. These results could provide the basis for a rational approach to ameliorate the effects of Huntington's disease...
  39. pmc A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease
    Maria Björkqvist
    Neuronal Survival Unit, Department of Experimental Medical Sciences, Wallenberg Neuroscience Center, Lund University, S 221 00 Lund, Sweden
    J Exp Med 205:1869-77. 2008
    ..Collectively, our data suggest parallel central nervous system and peripheral pathogenic pathways of immune activation in HD...
  40. ncbi request reprint Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease
    Maria Björkqvist
    Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, BMC A10, Lund, Sweden
    Hum Mol Genet 15:1713-21. 2006
    ..This progressive increase in cortisol may contribute to the clinical symptoms, such as muscular wasting, mood changes and some of the cognitive deficits that occur in HD...
  41. ncbi request reprint Arfaptin 2 regulates the aggregation of mutant huntingtin protein
    Peter J Peters
    Division of Tumor Biology, Netherlands Cancer Institute, Amsterdam, The Netherlands
    Nat Cell Biol 4:240-5. 2002
    ..Our data suggest that arfaptin 2 is involved in regulating huntingtin protein aggregation, possibly by impairing proteasome function...
  42. ncbi request reprint Complex alteration of NMDA receptors in transgenic Huntington's disease mouse brain: analysis of mRNA and protein expression, plasma membrane association, interacting proteins, and phosphorylation
    Ruth Luthi-Carter
    MassGeneral Institute for Neurodegenerative Diseases, B114 2000, 114 16th Street, Charlestown, MA 02129 4404, USA
    Neurobiol Dis 14:624-36. 2003
    ..Taken together, these data demonstrate multiple levels of NMDA receptor dysregulation, including abnormalities in mRNA expression levels, receptor stoichiometry, protein phosphorylation, and receptor trafficking...
  43. ncbi request reprint Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice
    Paolo Guidetti
    Maryland Psychiatric Research Center, University of Maryland School of Medicine, Baltimore, MD 21228, USA
    Neurobiol Dis 23:190-7. 2006
    ....
  44. ncbi request reprint A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease
    Todd W Miller
    Division of Genetic Disorders Wadsworth Center, New York State Department of Health, and Department of Biomedical Sciences, University at Albany, NY 12208, USA
    Neurobiol Dis 19:47-56. 2005
    ..Intrabodies may be used as potential curative agents, and as drug discovery tools, for HD and other misfolded protein disorders...
  45. ncbi request reprint Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy
    Tsz M Tsang
    Biological Chemistry, Biomedical Sciences Division, Faculty of Medicine, Imperial College, London, SW7 2AZ, United Kingdom
    J Proteome Res 5:483-92. 2006
    ..Clear differentiation of R6/2 and wild-type mice was also obtained for urine and blood metabolite profiles that may have applicability for monitoring HD in human populations...
  46. ncbi request reprint Mutant huntingtin's effects on striatal gene expression in mice recapitulate changes observed in human Huntington's disease brain and do not differ with mutant huntingtin length or wild-type huntingtin dosage
    Alexandre Kuhn
    Ecole Polytechnique Federale de Lausanne EPFL, 1015 Lausanne, Switzerland
    Hum Mol Genet 16:1845-61. 2007
    ....
  47. ncbi request reprint Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease
    Eugenia Papalexi
    Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, BMC A10, 221 84 Lund, Sweden
    Eur J Neurosci 22:1541-6. 2005
    ..Gonadotropic hormone replacement did not mitigate weight loss or restore motor function in R6/2 males...
  48. ncbi request reprint Global changes to the ubiquitin system in Huntington's disease
    Eric J Bennett
    Department of Biological Sciences, Stanford University, Stanford, California 94305, USA
    Nature 448:704-8. 2007
    ..Thus, HD is linked to global changes in the ubiquitin system to a much greater extent than previously recognized...
  49. ncbi request reprint Increased metabolism in the R6/2 mouse model of Huntington's disease
    Jorien M M van der Burg
    Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Lund University BMC A10, Lund, Sweden
    Neurobiol Dis 29:41-51. 2008
    ..Together, these data demonstrate that weight loss in R6/2 mice is associated with increased metabolism and changes in several weight-regulating factors...
  50. ncbi request reprint Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease
    Andreas Weiss
    Neuroscience Discovery, Novartis Institutes for BioMedical Research, Basel, Switzerland
    J Neurochem 104:846-58. 2008
    ....
  51. pmc A potent small molecule inhibits polyglutamine aggregation in Huntington's disease neurons and suppresses neurodegeneration in vivo
    Xiaoqian Zhang
    Department of Biochemistry, Boston University Medical School, Boston, MA 02118, USA
    Proc Natl Acad Sci U S A 102:892-7. 2005
    ..The aggregation inhibitors identified in this screen represent four primary chemical scaffolds and are strong lead compounds for the development of therapeutics for human polyQ diseases...
  52. ncbi request reprint Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction
    Ruben Smith
    Neuronal Survival Unit, Wallenberg Neuroscience Center, Department of Experimental Medical Science, Lund University, BMC A10, 221 84 Lund, Sweden
    Neurobiol Dis 20:673-84. 2005
    ..Our results indicate that a decrease in mRNA levels underlie the depletion of protein levels of rabphilin 3A, and we suggest that this reduction may be involved in causing impaired synaptic transmission in R6/1 mice...
  53. ncbi request reprint Alterations in the mouse and human proteome caused by Huntington's disease
    Claus Zabel
    Institut fur Humangenetik, Universitatsklinikum Charite, 13353 Berlin, Germany
    Mol Cell Proteomics 1:366-75. 2002
    ..Maintaining alpha1-antitrypsin and alphaB-crystallin availability during the course of Huntington's disease might prevent neuronal cell death and therefore could be useful in delaying the disease progression...
  54. ncbi request reprint Polyglutamine expansion of huntingtin impairs its nuclear export
    Jonathan Cornett
    Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Atlanta, Georgia 30322, USA
    Nat Genet 37:198-204. 2005
    ..These results suggest that Tpr has a role in the nuclear export of N-terminal htt and that polyQ expansion reduces this nuclear export to cause the nuclear accumulation of htt...
  55. ncbi request reprint Proteomic profiling of plasma in Huntington's disease reveals neuroinflammatory activation and biomarker candidates
    Annette Dalrymple
    Proteome Sciences plc, Cobham, Surrey, United Kingdom
    J Proteome Res 6:2833-40. 2007
    ..Proteins of interest were evaluated using immunoblotting and ELISA in plasma from 2 populations, CSF and R6/2 mice. The identified proteins demonstrate neuroinflammation in HD and warrant further investigation as possible biomarkers...
  56. ncbi request reprint Biomarkers for neurodegenerative diseases
    Susie M D Henley
    Dementia Research Centre, Institute of Neurology, University College London, London, UK
    Curr Opin Neurol 18:698-705. 2005
    ..This review summarizes the field of biomarker research in the major neurodegenerative diseases...