List of works - Amber L Southwell

A fully humanized transgenic mouse model of Huntington disease.

scientific article

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles

scientific article published on 18 January 2017

Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background.

scientific article published on January 2012

Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients

scientific article

An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes

scientific article

Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease.

scientific article

Antibody Therapy in Neurodegenerative Disease ⬇️

scientific article published on January 1, 2010

Assessment of motor balance and coordination in mice using the balance beam

scientific article

Bidirectional control of postsynaptic density-95 (PSD-95) clustering by Huntingtin

scientific article published on 17 December 2013

Biophysical and biological characterization of hairpin and molecular beacon RNase H active antisense oligonucleotides.

scientific article

Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo.

scientific article published in January 2012

Design, Characterization, and Lead Selection of Therapeutic miRNAs Targeting Huntingtin for Development of Gene Therapy for Huntington's Disease.

scientific article

Enhanced immune response to MMP3 stimulation in microglia expressing mutant huntingtin

scientific article published on 23 March 2016

Evolution and divergence of sodium channel genes in vertebrates.

scientific article published in June 2001

GABA transporter deficiency causes tremor, ataxia, nervousness, and increased GABA-induced tonic conductance in cerebellum.

scientific article published in March 2005

Gene therapy in mouse models of huntington disease.

scientific article

HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.

scientific article

HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response.

scientific article published on 10 February 2014

Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease.

scientific article published on 16 October 2012

Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry.

scientific article published on 23 July 2015

In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides.

scientific article published on 7 August 2014

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

scientific article

Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease.

scientific article published on 19 August 2011

Personalized gene silencing therapeutics for Huntington disease.

scientific article published on 11 April 2014

Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1

scientific article

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

scientific article

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease

scientific article published on 6 March 2018

Striatal synaptic dysfunction and hippocampal plasticity deficits in the Hu97/18 mouse model of Huntington disease

scientific article

Sudden death due to paralysis and synaptic and behavioral deficits when Hip14/Zdhhc17 is deleted in adult mice.

scientific article

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression

scientific article published on 15 July 2015

List of works by Amber L Southwell

A fully humanized transgenic mouse model of Huntington disease.

A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles

Age-Dependent Resistance to Excitotoxicity in Htt CAG140 Mice and the Effect of Strain Background.

Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients

An enhanced Q175 knock-in mouse model of Huntington disease with higher mutant huntingtin levels and accelerated disease phenotypes

Anti-semaphorin 4D immunotherapy ameliorates neuropathology and some cognitive impairment in the YAC128 mouse model of Huntington disease.

Antibody Therapy in Neurodegenerative Disease ⬇️

Assessment of motor balance and coordination in mice using the balance beam

Bidirectional control of postsynaptic density-95 (PSD-95) clustering by Huntingtin

Biophysical and biological characterization of hairpin and molecular beacon RNase H active antisense oligonucleotides.

Caspase-6-Resistant Mutant Huntingtin Does not Rescue the Toxic Effects of Caspase-Cleavable Mutant Huntingtin in vivo.

Design, Characterization, and Lead Selection of Therapeutic miRNAs Targeting Huntingtin for Development of Gene Therapy for Huntington's Disease.

Enhanced immune response to MMP3 stimulation in microglia expressing mutant huntingtin

Evolution and divergence of sodium channel genes in vertebrates.

GABA transporter deficiency causes tremor, ataxia, nervousness, and increased GABA-induced tonic conductance in cerebellum.

Gene therapy in mouse models of huntington disease.

HACE1 is essential for astrocyte mitochondrial function and influences Huntington disease phenotypes in vivo.

HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response.

Hip14l-deficient mice develop neuropathological and behavioural features of Huntington disease.

Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry.

In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides.

Marked differences in neurochemistry and aggregates despite similar behavioural and neuropathological features of Huntington disease in the full-length BACHD and YAC128 mice

Mice lacking caspase-2 are protected from behavioral changes, but not pathology, in the YAC128 model of Huntington disease.

Personalized gene silencing therapeutics for Huntington disease.

Perturbation with intrabodies reveals that calpain cleavage is required for degradation of huntingtin exon 1

Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease

Striatal synaptic dysfunction and hippocampal plasticity deficits in the Hu97/18 mouse model of Huntington disease

Sudden death due to paralysis and synaptic and behavioral deficits when Hip14/Zdhhc17 is deleted in adult mice.

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression