Search Results - "BROOKS, Simon P"
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Exercise attenuates neuropathology and has greater benefit on cognitive than motor deficits in the R6/1 Huntington's disease mouse model
Published in Experimental neurology (01-10-2013)“…Huntington's disease (HD) is a neurodegenerative disease caused by a mutation within the huntingtin gene that induces degeneration within the striatal nuclei,…”
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Comparison of mHTT Antibodies in Huntington's Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development
Published in PloS one (19-05-2016)“…Huntington's disease (HD) cellular pathology is characterised by the aggregation of mutant huntingtin (mHTT) protein into inclusion bodies. The present paper…”
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CTIP2-Regulated Reduction in PKA-Dependent DARPP32 Phosphorylation in Human Medium Spiny Neurons: Implications for Huntington Disease
Published in Stem cell reports (10-09-2019)“…The mechanisms underlying the selective degeneration of medium spiny neurons (MSNs) in Huntington disease (HD) remain largely unknown. CTIP2, a transcription…”
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A Longitudinal Operant Assessment of Cognitive and Behavioural Changes in the HdhQ111 Mouse Model of Huntington's Disease
Published in PloS one (01-10-2016)“…Huntington's disease (HD) is characterised by motor symptoms which are often preceded by cognitive and behavioural changes, that can significantly contribute…”
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Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD
Published in PloS one (01-12-2015)“…Huntington's disease is a neurodegenerative disorder characterised primarily by motor abnormalities, and is caused by an expanded polyglutamine repeat in the…”
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Loss of CRMP2 O-GlcNAcylation leads to reduced novel object recognition performance in mice
Published in Open biology (01-11-2019)“…O-GlcNAcylation is an abundant post-translational modification in the nervous system, linked to both neurodevelopmental and neurodegenerative disease. However,…”
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The Effect of Tissue Preparation and Donor Age on Striatal Graft Morphology in the Mouse
Published in Cell transplantation (01-02-2018)“…Huntington's disease (HD) is a progressive neurodegenerative disease in which striatal medium spiny neurons (MSNs) are lost. Neuronal replacement therapies aim…”
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Correction: Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD
Published in PloS one (2016)“…[This corrects the article DOI: 10.1371/journal.pone.0144864.]…”
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Basal Mitophagy Occurs Independently of PINK1 in Mouse Tissues of High Metabolic Demand
Published in Cell metabolism (06-02-2018)“…Dysregulated mitophagy has been linked to Parkinson's disease (PD) due to the role of PTEN-induced kinase 1 (PINK1) in mediating depolarization-induced…”
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X-linked cataract and Nance-Horan syndrome are allelic disorders
Published in Human molecular genetics (15-07-2009)“…Nance-Horan syndrome (NHS) is an X-linked developmental disorder characterized by congenital cataract, dental anomalies, facial dysmorphism and, in some cases,…”
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Motor Assessment in Huntington's Disease Mice
Published in Methods in molecular biology (Clifton, N.J.) (01-01-2018)“…Motor deficits are a characteristic consequence of striatal damage, whether induced by experimental lesions, or in genetic models of Huntington's disease…”
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The utilisation of operant delayed matching and non-matching to position for probing cognitive flexibility and working memory in mouse models of Huntington's disease
Published in Journal of neuroscience methods (30-05-2016)“…•We compared delayed matching and non-matching to position (DMTP and DNMTP) tasks in two different operant apparatus, the 9-hole operant apparatus…”
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Optimising Golgi–Cox staining for use with perfusion-fixed brain tissue validated in the zQ175 mouse model of Huntington's disease
Published in Journal of neuroscience methods (30-05-2016)“…•A modified reliable Golgi–Cox method is described.•It is inexpensive and can be performed in any neuroscience lab.•Perfusion–fixation with 4% paraformaldehyde…”
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Tests to assess motor phenotype in mice: a user's guide
Published in Nature reviews. Neuroscience (01-07-2009)“…Key Points The identification of motor abnormalities in mouse models of disease is dependent on the choice of an appropriate behavioural test(s). The utility…”
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Cognitive training modifies disease symptoms in a mouse model of Huntington's disease
Published in Experimental neurology (01-08-2016)“…Huntington's disease (HD) is an incurable neurodegenerative disorder which causes a triad of motor, cognitive and psychiatric disturbances. Cognitive…”
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Phosphorylation of Parkin at serine 65 is essential for its activation in vivo
Published in Open biology (07-11-2018)“…Mutations in PINK1 and Parkin result in autosomal recessive Parkinson's disease (PD). Cell culture and studies have elaborated the PINK1-dependent regulation…”
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Comparative analysis of pathology and behavioural phenotypes in mouse models of Huntington's disease
Published in Brain research bulletin (01-06-2012)“…Highlights ► The rationale for the longitudinal HD mouse characterisation is described. ► Longitudinal behavioural, genetic and neuropathological methods are…”
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Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice
Published in Brain research bulletin (01-06-2012)“…Highlights ► The authors sought to longitudinally characterise the nature, severity and development of behavioural abnormalities in the R6/1 (C57BL/6J) mouse…”
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Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 Huntington's disease transgenic mice
Published in Brain research bulletin (01-06-2012)“…Highlights ► Characterization of disease neuropathology in the (C57BL/6J) YAC128 mouse line. ► The YAC128 mice showed a greater density of aggregates in the…”
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Light and electron microscopic characterization of the evolution of cellular pathology in the R6/1 Huntington's disease transgenic mice
Published in Brain research bulletin (01-06-2012)“…Highlights ► The study aimed to characterize anatomically the disease progression in the R6/1 mouse line using S830 and GFAP immunohistochemistry, stereology…”
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