Simon P. Brooks
YOU?
Author Swipe
View article: Complex cognitive and motivational deficits precede motor dysfunction in the zQ175 (190 CAG repeat) Huntington's disease model
Complex cognitive and motivational deficits precede motor dysfunction in the zQ175 (190 CAG repeat) Huntington's disease model Open
Huntington's disease (HD) is a progressive, inherited neurodegenerative disorder characterised by motor, cognitive, and neuropsychiatric dysfunction for which several mouse models have been developed. Knock-in models, such as zQ175, retain…
View article: Endogenous LRRK2 and PINK1 function in a convergent neuroprotective ciliogenesis pathway in the brain
Endogenous LRRK2 and PINK1 function in a convergent neuroprotective ciliogenesis pathway in the brain Open
Mutations in Leucine-rich repeat kinase 2 (LRRK2) and PTEN-induced kinase 1 (PINK1) are associated with familial Parkinson’s disease (PD). LRRK2 phosphorylates Rab guanosine triphosphatase (GTPases) within the Switch II domain while PINK1 …
View article: Endogenous LRRK2 and PINK1 function in a convergent neuroprotective ciliogenesis pathway in the brain
Endogenous LRRK2 and PINK1 function in a convergent neuroprotective ciliogenesis pathway in the brain Open
Mutations in LRRK2 and PINK1 are associated with familial Parkinson’s disease (PD). LRRK2 phosphorylates Rab GTPases within the Switch II domain whilst PINK1 directly phosphorylates Parkin and ubiquitin and indirectly induces phosphorylati…
View article: Loss of CRMP2 O-GlcNAcylation leads to reduced novel object recognition performance in mice
Loss of CRMP2 O-GlcNAcylation leads to reduced novel object recognition performance in mice Open
O-GlcNAcylation is an abundant post-translational modification in the nervous system, linked to both neurodevelopmental and neurodegenerative disease. However, the mechanistic links between these phenotypes and site-specific O-GlcNAcylatio…
View article: Modulation of ERK1/MAPK3 potentiates ERK nuclear signalling, facilitates neuronal cell survival and improves memory in mouse models of neurodegenerative disorders
Modulation of ERK1/MAPK3 potentiates ERK nuclear signalling, facilitates neuronal cell survival and improves memory in mouse models of neurodegenerative disorders Open
Cell signalling mechanisms are central to neuronal activity and their dysregulation may lead to neurodegenerative processes and associated cognitive decline. So far, a major effort has been directed toward the dissection of disease specifi…
View article: Phosphorylation of Parkin at serine 65 is essential for its activation<i>in vivo</i>
Phosphorylation of Parkin at serine 65 is essential for its activation<i>in vivo</i> Open
Mutations in PINK1 and Parkin result in autosomal recessive Parkinson's disease (PD). Cell culture and in vitro studies have elaborated the PINK1-dependent regulation of Parkin and defined how this dyad orchestrates the elimination of dama…
View article: The Effect of Tissue Preparation and Donor Age on Striatal Graft Morphology in the Mouse
The Effect of Tissue Preparation and Donor Age on Striatal Graft Morphology in the Mouse Open
Huntington's disease (HD) is a progressive neurodegenerative disease in which striatal medium spiny neurons (MSNs) are lost. Neuronal replacement therapies aim to replace MSNs through striatal transplantation of donor MSN progenitors, whic…
View article: Supplementary Table 2 from Phosphorylation of Parkin at serine 65 is essential for its activation <i>in vivo</i>
Supplementary Table 2 from Phosphorylation of Parkin at serine 65 is essential for its activation <i>in vivo</i> Open
Mutations in PINK1 and Parkin result in autosomal recessive Parkinson's disease (PD). Cell culture and in vitro studies have elaborated the PINK1-dependent regulation of Parkin and defined how this dyad orchestrates the elimination of dama…
View article: Supplementary material from "Phosphorylation of Parkin at serine 65 is essential for its activation <i>in vivo</i>"
Supplementary material from "Phosphorylation of Parkin at serine 65 is essential for its activation <i>in vivo</i>" Open
Mutations in PINK1 and Parkin result in autosomal recessive Parkinson's disease (PD). Cell culture and in vitro studies have elaborated the PINK1-dependent regulation of Parkin and defined how this dyad orchestrates the elimination of dama…
View article: Correction: Corrigendum: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington’s disease
Correction: Corrigendum: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington’s disease Open
Scientific Reports 7: Article number: 41570; published online: 08 February 2017; updated: 28 March 2017 This Article contains a typographical error in the Methods section, under the subheading “Library construction, RNA Sequencing and RNAS…
View article: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington’s disease
Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.HttQ111/+ model of Huntington’s disease Open
We investigated the appearance and progression of disease-relevant signs in the B6. Htt Q111/+ mouse, a genetically precise model of the mutation that causes Huntington’s disease (HD). We find that B6. Htt Q111/+ mice are healthy, show no …
View article: Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.<i>Htt</i><sup><i>Q111/+</i></sup>model of Huntington’s disease
Motivational, proteostatic and transcriptional deficits precede synapse loss, gliosis and neurodegeneration in the B6.<i>Htt</i><sup><i>Q111/+</i></sup>model of Huntington’s disease Open
We investigated the appearance and progression of disease-relevant signs in the B6. Htt Q111/+ mouse, a genetically precise model of the mutation that causes Huntington’s disease (HD). We find that B6. Htt Q111/+ mice are healthy, show no …
View article: Correction: Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD
Correction: Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD Open
[This corrects the article DOI: 10.1371/journal.pone.0144864.].
View article: A Longitudinal Operant Assessment of Cognitive and Behavioural Changes in the HdhQ111 Mouse Model of Huntington’s Disease
A Longitudinal Operant Assessment of Cognitive and Behavioural Changes in the HdhQ111 Mouse Model of Huntington’s Disease Open
Huntington's disease (HD) is characterised by motor symptoms which are often preceded by cognitive and behavioural changes, that can significantly contribute to disease burden for people living with HD. Numerous knock-in mouse models of HD…
View article: A Longitudinal Motor Characterisation of the HdhQ111 Mouse Model of Huntington’s Disease
A Longitudinal Motor Characterisation of the HdhQ111 Mouse Model of Huntington’s Disease Open
The HdhQ111 heterozygous mouse demonstrates a subtle and progressive motor phenotype that begins at 9 months of age. This mouse model represents an early disease stage and would be ideal for testing therapeutic strategies that require elon…
View article: Comparison of mHTT Antibodies in Huntington’s Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development
Comparison of mHTT Antibodies in Huntington’s Disease Mouse Models Reveal Specific Binding Profiles and Steady-State Ubiquitin Levels with Disease Development Open
Huntington's disease (HD) cellular pathology is characterised by the aggregation of mutant huntingtin (mHTT) protein into inclusion bodies. The present paper compared the sensitivity of five widely used mHTT antibodies (S830; MW8; EM48; 1C…
View article: Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD
Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdhQ111 Model of HD Open
Huntington's disease is a neurodegenerative disorder characterised primarily by motor abnormalities, and is caused by an expanded polyglutamine repeat in the huntingtin protein. Huntingtin dynamically shuttles between subcellular compartme…
View article: Optimising Golgi–Cox staining for use with perfusion-fixed brain tissue validated in the zQ175 mouse model of Huntington's disease
Optimising Golgi–Cox staining for use with perfusion-fixed brain tissue validated in the zQ175 mouse model of Huntington's disease Open
The present study introduces a robust reproducible and inexpensive staining method for identifying neuronal morphological changes in the post fixed mouse brain, and is suitable for assessing changes in cell morphology in models of neurodeg…
View article: In Vivo MRI Evidence that Neuropathology is Attenuated by Cognitive Enrichment in the Yac128 Huntington’s Disease Mouse Model
In Vivo MRI Evidence that Neuropathology is Attenuated by Cognitive Enrichment in the Yac128 Huntington’s Disease Mouse Model Open
We provide in vivo evidence of a beneficial effect of environmental enrichment on neuropathology and motor function in a HD mouse model. This demonstrates the efficacy of MRI in a model of HD and provides the basis for an in-vivo non-destr…
View article: Additional file 2: Table S2. of Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntingtonâ s disease are not reflected in mutant Huntingtin inclusion prevalence
Additional file 2: Table S2. of Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntingtonâ s disease are not reflected in mutant Huntingtin inclusion prevalence Open
Over-representation analysis of the gene expression differences with age in YAC128 and WT striata (XLS 116 kb)
View article: Additional file 5: Table S4. of Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntingtonâ s disease are not reflected in mutant Huntingtin inclusion prevalence
Additional file 5: Table S4. of Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntingtonâ s disease are not reflected in mutant Huntingtin inclusion prevalence Open
Over-representation analysis of the 2557 genes nominally significantly differentially expressed between YAC128 and WT striata (XLS 61 kb)