Gillian P Bates

Gillian P Bates

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Gillian P Bates

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Genetic deletion of S6k1 does not rescue the phenotypic deficits observed in the R6/2 mouse model of Huntington's disease.

Sci Rep 2019 Nov 6;9(1):16133. Epub 2019 Nov 6.

MRC London Institute of Medical Sciences, Imperial College London, Hammersmith Campus, London, W12 0NN, UK.

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http://dx.doi.org/10.1038/s41598-019-52391-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6834565PMC
November 2019

Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay.

Sci Rep 2019 Nov 6;9(1):16137. Epub 2019 Nov 6.

Huntington's Disease Centre, Department of Neurodegenerative Disease and UK Dementia Research Institute at UCL, Queen Square Institute of Neurology, University College London, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1038/s41598-019-52411-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6834638PMC
November 2019

Inhibition of tumour necrosis factor alpha in the R6/2 mouse model of Huntington's disease by etanercept treatment.

Sci Rep 2019 May 10;9(1):7202. Epub 2019 May 10.

Huntington's Disease Centre, Department of Neurodegenerative Disease and UK Dementia Research Institute, UCL Queen Square Institute of Neurology, University College London, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1038/s41598-019-43627-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6510744PMC
May 2019

Mouse Models of Huntington's Disease.

Methods Mol Biol 2018 ;1780:97-120

Department of Neurodegenerative Disease, Huntington's Disease Centre and Dementia Research Institute, University College London Institute of Neurology, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1007/978-1-4939-7825-0_6DOI Listing
February 2019

Regulatory mechanisms of incomplete huntingtin mRNA splicing.

Nat Commun 2018 09 27;9(1):3955. Epub 2018 Sep 27.

UCL Huntington's Disease Centre, Department of Neurodegenerative Disease and Dementia Research Institute, UCL Institute of Neurology, University College London, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1038/s41467-018-06281-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6160442PMC
September 2018

RNA Related Pathology in Huntington's Disease.

Adv Exp Med Biol 2018 ;1049:85-101

Sobell Department of Motor Neuroscience, UCL Institute of Neurology, Queen Square, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1007/978-3-319-71779-1_4DOI Listing
July 2018

In vivo neutralization of the protagonist role of macrophages during the chronic inflammatory stage of Huntington's disease.

Sci Rep 2018 07 30;8(1):11447. Epub 2018 Jul 30.

Huntington's Disease Centre, Department of Neurodegenerative Disease and Dementia Research Institute, UCL Institute of Neurology, University College London, London, WC1N 3BG, UK.

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http://dx.doi.org/10.1038/s41598-018-29792-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6065433PMC
July 2018

Stall in Canonical Autophagy-Lysosome Pathways Prompts Nucleophagy-Based Nuclear Breakdown in Neurodegeneration.

Curr Biol 2017 Dec 22;27(23):3626-3642.e6. Epub 2017 Nov 22.

Department of Basic and Clinical Neuroscience, King's College London, 125 Coldharbour Lane, SE5 9NU London, UK. Electronic address:

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http://dx.doi.org/10.1016/j.cub.2017.10.054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5723708PMC
December 2017

HSF1-dependent and -independent regulation of the mammalian in vivo heat shock response and its impairment in Huntington's disease mouse models.

Sci Rep 2017 10 2;7(1):12556. Epub 2017 Oct 2.

UCL Huntington's Disease Centre, Sobell Department of Motor Neuroscience, UCL Institute of Neurology, University College London, London, United Kingdom.

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http://dx.doi.org/10.1038/s41598-017-12897-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5624871PMC
October 2017

Disruption to schizophrenia-associated gene Fez1 in the hippocampus of HDAC11 knockout mice.

Sci Rep 2017 09 19;7(1):11900. Epub 2017 Sep 19.

UCL Huntington's Disease Centre, Sobell Department of Motor Neuroscience, UCL Institute of Neurology, University College London, London, United Kingdom.

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http://dx.doi.org/10.1038/s41598-017-11630-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5605701PMC
September 2017

The pathogenic exon 1 HTT protein is produced by incomplete splicing in Huntington's disease patients.

Sci Rep 2017 05 2;7(1):1307. Epub 2017 May 2.

UCL Huntington's Disease Centre, Sobell Department of Motor Neuroscience, UCL Institute of Neurology, University College London, London, United Kingdom.

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http://dx.doi.org/10.1038/s41598-017-01510-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5431000PMC
May 2017

Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington's Disease.

J Huntingtons Dis 2016 12;5(4):343-346

Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, London, UK.

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http://dx.doi.org/10.3233/JHD-160217DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5181660PMC
December 2016

UBQLN2 Mediates Autophagy-Independent Protein Aggregate Clearance by the Proteasome.

Cell 2016 Aug 28;166(4):935-949. Epub 2016 Jul 28.

Institute of Molecular, Cell and Systems Biology, College of Medical, Veterinary and Life Sciences, Davidson Building, Henry Wellcome Lab of Cell Biology, University of Glasgow, G12 8QQ Glasgow, UK; The MRC Protein Phosphorylation and Ubiquitylation Unit, The Sir James Black Centre, College of Life Sciences, University of Dundee, Dow Street, Dundee DD1 5EH, Scotland. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2016.07.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5003816PMC
August 2016

In Vivo Profiling Reveals a Competent Heat Shock Response in Adult Neurons: Implications for Neurodegenerative Disorders.

PLoS One 2015 2;10(7):e0131985. Epub 2015 Jul 2.

Dept. Medical and Molecular Genetics, King's College London, 8th Floor Tower Wing, Guy's Hospital, Great Maze Pond, London, SE1 9RT, United Kingdom.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0131985PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4489736PMC
April 2016

Treating the whole body in Huntington's disease.

Lancet Neurol 2015 Nov;14(11):1135-42

Department of Neurodegenerative Disease, University College London Institute of Neurology, London, UK.

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http://dx.doi.org/10.1016/S1474-4422(15)00177-5DOI Listing
November 2015

Huntington disease.

Nat Rev Dis Primers 2015 04 23;1:15005. Epub 2015 Apr 23.

Department of Neurodegenerative Disease, University College London Institute of Neurology, Queen Square, London WC1N 3BG, UK.

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http://dx.doi.org/10.1038/nrdp.2015.5DOI Listing
April 2015

A common gene expression signature in Huntington's disease patient brain regions.

BMC Med Genomics 2014 Oct 30;7:60. Epub 2014 Oct 30.

Department of Medical and Molecular Genetics, King's College London, London, SE1 9RT, UK.

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http://dx.doi.org/10.1186/s12920-014-0060-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4219025PMC
October 2014

Correlations of behavioral deficits with brain pathology assessed through longitudinal MRI and histopathology in the R6/1 mouse model of Huntington's disease.

PLoS One 2013 19;8(12):e84726. Epub 2013 Dec 19.

King's College London, Institute of Psychiatry, Department of Neuroscience, London, United Kingdom ; University of Pittsburgh, Department of Radiology, McGowan Institute for Regenerative Medicine, Pittsburgh, Pennsylvania, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0084726PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3868608PMC
July 2014

Contesting the dogma of an age-related heat shock response impairment: implications for cardiac-specific age-related disorders.

Hum Mol Genet 2014 Jul 19;23(14):3641-56. Epub 2014 Feb 19.

Department Medical and Molecular Genetics, King's College London, 8th Floor Tower Wing, Guy's Hosptial, Great Maze Pond, London SE1 9RT, UK

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http://dx.doi.org/10.1093/hmg/ddu073DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4065144PMC
July 2014

Novel isoforms of heat shock transcription factor 1, HSF1γα and HSF1γβ, regulate chaperone protein gene transcription.

J Biol Chem 2014 Jul 22;289(29):19894-906. Epub 2014 May 22.

From the Department of Medical and Molecular Genetics, King's College London, London SE1 9RT, United Kingdom and

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http://dx.doi.org/10.1074/jbc.M114.570739DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4106310PMC
July 2014

Dynamic recruitment of active proteasomes into polyglutamine initiated inclusion bodies.

FEBS Lett 2014 Jan 26;588(1):151-9. Epub 2013 Nov 26.

Department of Cell Biology and Histology, Academic Medical Center, University of Amsterdam, Meibergdreef 15, 1105 AZ, The Netherlands. Electronic address:

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http://dx.doi.org/10.1016/j.febslet.2013.11.023DOI Listing
January 2014

Aberrantly spliced HTT, a new player in Huntington's disease pathogenesis.

RNA Biol 2013 Nov 11;10(11):1647-52. Epub 2013 Oct 11.

Koch Institute for Integrative Cancer Research; Massachusetts Institute of Technology; Cambridge, MA USA.

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http://dx.doi.org/10.4161/rna.26706DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3907474PMC
November 2013

Targeting H3K4 trimethylation in Huntington disease.

Proc Natl Acad Sci U S A 2013 Aug 19;110(32):E3027-36. Epub 2013 Jul 19.

Department of Psychiatry and Human Behavior and UCI Institute of Memory Impairments and Neurological Disorders, University of California, Irvine, CA 92697, USA.

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http://dx.doi.org/10.1073/pnas.1311323110DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3740882PMC
August 2013

Suppression of protein aggregation by chaperone modification of high molecular weight complexes.

Brain 2012 Apr 6;135(Pt 4):1180-96. Epub 2012 Mar 6.

Department of Medical and Molecular Genetics, King’s College London, London SE1 9RT, UK.

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http://dx.doi.org/10.1093/brain/aws022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3326252PMC
April 2012

Gastrointestinal dysfunction contributes to weight loss in Huntington's disease mice.

Neurobiol Dis 2011 Oct 23;44(1):1-8. Epub 2011 May 23.

Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Lund University, BMC A10, Lund, Sweden.

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http://dx.doi.org/10.1016/j.nbd.2011.05.006DOI Listing
October 2011

The importance of integrating basic and clinical research toward the development of new therapies for Huntington disease.

J Clin Invest 2011 Feb 1;121(2):476-83. Epub 2011 Feb 1.

CHDI Management Inc./CHDI Foundation Inc., 6080 Center Drive, Suite 100, Los Angeles, California 90046, USA.

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http://dx.doi.org/10.1172/JCI45364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3026740PMC
February 2011

CalDAG-GEFI down-regulation in the striatum as a neuroprotective change in Huntington's disease.

Hum Mol Genet 2010 May 10;19(9):1756-65. Epub 2010 Feb 10.

McGovern Institute for Brain Research, MIT, 43 Vassar Street, Building 46-6133, Cambridge, MA, USA.

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http://dx.doi.org/10.1093/hmg/ddq055DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2850620PMC
May 2010

Identical oligomeric and fibrillar structures captured from the brains of R6/2 and knock-in mouse models of Huntington's disease.

Hum Mol Genet 2010 Jan;19(1):65-78

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.

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http://dx.doi.org/10.1093/hmg/ddp467DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2792149PMC
January 2010

Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease.

PLoS One 2009 Nov 30;4(11):e8025. Epub 2009 Nov 30.

Department of Medical and Molecular Genetics, King's College London School of Medicine, London, United Kingdom.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0008025PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2778556PMC
November 2009

The polyubiquitin Ubc gene modulates histone H2A monoubiquitylation in the R6/2 mouse model of Huntington's disease.

J Cell Mol Med 2009 Aug;13(8B):2645-57

King's College London School of Medicine, Department of Medical and Molecular Genetics, King's College London, London, United Kingdom.

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http://dx.doi.org/10.1111/j.1582-4934.2008.00543.xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2892477PMC
August 2009

The ubiquitin-proteasome reporter GFPu does not accumulate in neurons of the R6/2 transgenic mouse model of Huntington's disease.

PLoS One 2009 8;4(4):e5128. Epub 2009 Apr 8.

Department of Medical and Molecular Genetics, King's College London School of Medicine, King's College London, London, United Kingdom.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0005128PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2662425PMC
July 2009

Optimisation of region-specific reference gene selection and relative gene expression analysis methods for pre-clinical trials of Huntington's disease.

Mol Neurodegener 2008 Oct 27;3:17. Epub 2008 Oct 27.

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.

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http://dx.doi.org/10.1186/1750-1326-3-17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2584034PMC
October 2008

DNA instability in postmitotic neurons.

Proc Natl Acad Sci U S A 2008 Mar 25;105(9):3467-72. Epub 2008 Feb 25.

Department of Medical and Molecular Genetics, King's College London School of Medicine, London SE1 9RT, United Kingdom.

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http://dx.doi.org/10.1073/pnas.0800048105DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2265187PMC
March 2008

Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.

J Neurochem 2008 Feb 6;104(3):846-58. Epub 2007 Nov 6.

Neuroscience Discovery, Novartis Institutes for BioMedical Research, Basel, Switzerland.

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http://dx.doi.org/10.1111/j.1471-4159.2007.05032.xDOI Listing
February 2008

Increased metabolism in the R6/2 mouse model of Huntington's disease.

Neurobiol Dis 2008 Jan 24;29(1):41-51. Epub 2007 Aug 24.

Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, Lund University BMC A10, Lund, Sweden.

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http://dx.doi.org/10.1016/j.nbd.2007.07.029DOI Listing
January 2008

Global changes to the ubiquitin system in Huntington's disease.

Nature 2007 Aug;448(7154):704-8

Department of Biological Sciences, Stanford University, Stanford, California 94305, USA.

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http://dx.doi.org/10.1038/nature06022DOI Listing
August 2007

Hsp27 overexpression in the R6/2 mouse model of Huntington's disease: chronic neurodegeneration does not induce Hsp27 activation.

Hum Mol Genet 2007 May 14;16(9):1078-90. Epub 2007 Mar 14.

Department of Medical and Molecular Genetics, King's College London, School of Medicine, London SE1 9RT, UK.

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http://dx.doi.org/10.1093/hmg/ddm057DOI Listing
May 2007

The Hdh(Q150/Q150) knock-in mouse model of HD and the R6/2 exon 1 model develop comparable and widespread molecular phenotypes.

Brain Res Bull 2007 Apr 5;72(2-3):83-97. Epub 2006 Dec 5.

Department of Medical and Molecular Genetics, King's College London School of Medicine, London, UK.

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http://dx.doi.org/10.1016/j.brainresbull.2006.11.004DOI Listing
April 2007

Histone deacetylase inhibitors as therapeutics for polyglutamine disorders.

Nat Rev Neurosci 2006 Oct;7(10):784-96

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.

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http://dx.doi.org/10.1038/nrn1989DOI Listing
October 2006

Elevated brain 3-hydroxykynurenine and quinolinate levels in Huntington disease mice.

Neurobiol Dis 2006 Jul 12;23(1):190-7. Epub 2006 May 12.

Maryland Psychiatric Research Center, University of Maryland School of Medicine, Baltimore, MD 21228, USA.

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http://dx.doi.org/10.1016/j.nbd.2006.02.011DOI Listing
July 2006

Progressive alterations in the hypothalamic-pituitary-adrenal axis in the R6/2 transgenic mouse model of Huntington's disease.

Hum Mol Genet 2006 May 13;15(10):1713-21. Epub 2006 Apr 13.

Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, BMC A10, Lund, Sweden.

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http://dx.doi.org/10.1093/hmg/ddl094DOI Listing
May 2006

Metabolic characterization of the R6/2 transgenic mouse model of Huntington's disease by high-resolution MAS 1H NMR spectroscopy.

J Proteome Res 2006 Mar;5(3):483-92

Biological Chemistry, Biomedical Sciences Division, Faculty of Medicine, Imperial College, London, SW7 2AZ, United Kingdom.

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http://dx.doi.org/10.1021/pr050244oDOI Listing
March 2006

BIOMEDICINE: One Misfolded Protein Allows Others to Sneak By.

Authors:
Gillian P Bates

Science 2006 Mar;311(5766):1385-6

King's College London School of Medicine, London SE1 9RT, UK.

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http://dx.doi.org/10.1126/science.1125246DOI Listing
March 2006

Mouse models of triplet repeat diseases.

Mol Biotechnol 2006 Feb;32(2):147-58

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.

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http://dx.doi.org/10.1385/MB:32:2:147DOI Listing
February 2006

Evaluation of the benzothiazole aggregation inhibitors riluzole and PGL-135 as therapeutics for Huntington's disease.

Neurobiol Dis 2006 Jan 18;21(1):228-36. Epub 2005 Aug 18.

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.

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http://dx.doi.org/10.1016/j.nbd.2005.07.007DOI Listing
January 2006

Proteasome impairment does not contribute to pathogenesis in R6/2 Huntington's disease mice: exclusion of proteasome activator REGgamma as a therapeutic target.

Hum Mol Genet 2006 Jan 25;15(1):33-44. Epub 2005 Nov 25.

Department of Medical and Molecular Genetics, GKT School of Medicine, King's College London, UK.

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http://dx.doi.org/10.1093/hmg/ddi423DOI Listing
January 2006

Depletion of rabphilin 3A in a transgenic mouse model (R6/1) of Huntington's disease, a possible culprit in synaptic dysfunction.

Neurobiol Dis 2005 Dec 20;20(3):673-84. Epub 2005 Jun 20.

Neuronal Survival Unit, Wallenberg Neuroscience Center, Department of Experimental Medical Science, Lund University, BMC A10, 221 84 Lund, Sweden.

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http://dx.doi.org/10.1016/j.nbd.2005.05.008DOI Listing
December 2005

Biomarkers for neurodegenerative diseases.

Curr Opin Neurol 2005 Dec;18(6):698-705

Dementia Research Centre, Institute of Neurology, University College London, London, UK.

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http://dx.doi.org/10.1097/01.wco.0000186842.51129.cbDOI Listing
December 2005

History of genetic disease: the molecular genetics of Huntington disease - a history.

Authors:
Gillian P Bates

Nat Rev Genet 2005 10;6(10):766-73

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.

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http://dx.doi.org/10.1038/nrg1686DOI Listing
October 2005

Reduction of GnRH and infertility in the R6/2 mouse model of Huntington's disease.

Eur J Neurosci 2005 Sep;22(6):1541-6

Neuronal Survival Unit, Department of Experimental Medical Science, Wallenberg Neuroscience Center, BMC A10, 221 84 Lund, Sweden.

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http://dx.doi.org/10.1111/j.1460-9568.2005.04324.xDOI Listing
September 2005

A human single-chain Fv intrabody preferentially targets amino-terminal Huntingtin's fragments in striatal models of Huntington's disease.

Neurobiol Dis 2005 Jun-Jul;19(1-2):47-56

Division of Genetic Disorders Wadsworth Center, New York State Department of Health, and Department of Biomedical Sciences, University at Albany, NY 12208, USA.

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http://dx.doi.org/10.1016/j.nbd.2004.11.003DOI Listing
July 2005

Polyglutamine expansion of huntingtin impairs its nuclear export.

Nat Genet 2005 Feb 16;37(2):198-204. Epub 2005 Jan 16.

Department of Human Genetics, Emory University School of Medicine, 615 Michael Street, Atlanta, Georgia 30322, USA.

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http://dx.doi.org/10.1038/ng1503DOI Listing
February 2005

Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series.

EMBO Rep 2004 Oct;5(10):958-63

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.

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http://dx.doi.org/10.1038/sj.embor.7400250DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1299150PMC
October 2004

Mouse models of triplet repeat diseases.

Methods Mol Biol 2004 ;277:3-15

Department of Medical and Molecular Genetics, GKT School of Medicine, King's College, Guy's Hospital, London, UK.

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http://dx.doi.org/10.1385/1-59259-804-8:003DOI Listing
September 2004

Monitoring aggregate formation in organotypic slice cultures from transgenic mice.

Methods Mol Biol 2004 ;277:161-71

Department of Medical and Molecular Genetics, GKT School of Medicine, King's College, Guy's Hospital, London, UK.

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http://link.springer.com/10.1385/1-59259-804-8:161
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http://dx.doi.org/10.1385/1-59259-804-8:161DOI Listing
September 2004