Search Results - "Ross, Christopher A."
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Huntington's disease: from molecular pathogenesis to clinical treatment
Published in Lancet neurology (2011)“…Summary Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene, which encodes an…”
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Transcriptome-wide discovery of microRNA binding sites in human brain
Published in Neuron (Cambridge, Mass.) (22-01-2014)“…The orchestration of brain function requires complex gene regulatory networks that are modulated, in part, by microRNAs (miRNAs). These noncoding RNAs…”
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Mutant Huntingtin Disrupts the Nuclear Pore Complex
Published in Neuron (Cambridge, Mass.) (05-04-2017)“…Huntington’s disease (HD) is caused by an expanded CAG repeat in the Huntingtin (HTT) gene. The mechanism(s) by which mutant HTT (mHTT) causes disease is…”
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RAN Translation in Huntington Disease
Published in Neuron (Cambridge, Mass.) (18-11-2015)“…Huntington disease (HD) is caused by a CAG ⋅ CTG expansion in the huntingtin (HTT) gene. While most research has focused on the HTT polyGln-expansion protein,…”
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Synaptic dysregulation in a human iPS cell model of mental disorders
Published in Nature (London) (20-11-2014)“…Generation and neural differentiation of induced pluripotent stem cells (iPS cells) from patients enables new ways to investigate the cellular pathophysiology…”
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Huntington disease: natural history, biomarkers and prospects for therapeutics
Published in Nature reviews. Neurology (01-04-2014)“…Key Points No disease-modifying treatments are currently available for Huntington disease (HD), but clinical trials of potential compounds are imminent;…”
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Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor
Published in Neurobiology of disease (01-09-2019)“…Huntington's disease (HD) is a neurodegenerative disease caused by a CAG repeat expansion in the Huntingtin gene (HTT), translated into a Huntingtin protein…”
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What is the role of protein aggregation in neurodegeneration?
Published in Nature reviews. Molecular cell biology (01-11-2005)Get full text
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Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study
Published in Lancet neurology (01-12-2014)“…Summary Background Although the association between cytosine-adenine-guanine (CAG) repeat length and age at onset of Huntington's disease is well known,…”
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Abnormal degradation of the neuronal stress-protective transcription factor HSF1 in Huntington’s disease
Published in Nature communications (13-02-2017)“…Huntington’s Disease (HD) is a neurodegenerative disease caused by poly-glutamine expansion in the Htt protein, resulting in Htt misfolding and cell death…”
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Astrocytes generated from patient induced pluripotent stem cells recapitulate features of Huntington's disease patient cells
Published in Molecular brain (21-05-2012)“…Huntington's Disease (HD) is a devastating neurodegenerative disorder that clinically manifests as motor dysfunction, cognitive impairment and psychiatric…”
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Acute Kidney Injury Leads to Inflammation and Functional Changes in the Brain
Published in Journal of the American Society of Nephrology (01-07-2008)“…Although neurologic sequelae of acute kidney injury (AKI) are well described, the pathogenesis of acute uremic encephalopathy is poorly understood. This study…”
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Kinase activity of mutant LRRK2 mediates neuronal toxicity
Published in Nature neuroscience (01-10-2006)“…Mutations in the the leucine-rich repeat kinase-2 (LRRK2) gene cause autosomal-dominant Parkinson disease and some cases of sporadic Parkinson disease. Here we…”
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Post-translational modifications clustering within proteolytic domains decrease mutant huntingtin toxicity
Published in The Journal of biological chemistry (24-11-2017)“…Huntington's disease (HD) is caused in large part by a polyglutamine expansion within the huntingtin (Htt) protein. Post-translational modifications (PTMs)…”
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TBK1 phosphorylates mutant Huntingtin and suppresses its aggregation and toxicity in Huntington's disease models
Published in The EMBO journal (01-09-2020)“…Phosphorylation of the N‐terminal domain of the huntingtin (HTT) protein has emerged as an important regulator of its localization, structure, aggregation,…”
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Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets
Published in Nature medicine (01-01-2012)“…Huntington's disease is a neurodegenerative disease caused by the accumulation of mutant htt protein. Now, two groups led by Dimitri Krainc and Wenzhen Duan…”
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Curcumin protects against A53T alpha-synuclein-induced toxicity in a PC12 inducible cell model for Parkinsonism
Published in Pharmacological research (01-05-2011)“…Parkinson's disease (PD) is a progressive neurodegenerative movement disorder characterized by selective loss of dopaminergic neurons and the presence of Lewy…”
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Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis
Published in Cell cycle (Georgetown, Tex.) (15-05-2012)“…Huntington disease (HD) is a neurodegenerative disorder caused by an expansion of a polyglutamine repeat within the HD gene product, huntingtin. Huntingtin, a…”
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Targeting H3K4 trimethylation in Huntington disease
Published in Proceedings of the National Academy of Sciences - PNAS (06-08-2013)“…Transcriptional dysregulation is an early feature of Huntington disease (HD). We observed gene-specific changes in histone H3 lysine 4 trimethylation (H3K4me3)…”
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Parkinson's Disease-Associated Mutations in Leucine-Rich Repeat Kinase 2 Augment Kinase Activity
Published in Proceedings of the National Academy of Sciences - PNAS (15-11-2005)“…Mutations in the leucine-rich repeat kinase 2 gene (LRRK2) cause late-onset Parkinson's disease (PD) with a clinical appearance indistinguishable from…”
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