Cargando…

Early detection of exon 1 huntingtin aggregation in zQ175 brains by molecular and histological approaches

Huntingtin-lowering approaches that target huntingtin expression are a major focus for therapeutic intervention for Huntington’s disease. When the cytosine, adenine and guanine repeat is expanded, the huntingtin pre-mRNA is alternatively processed to generate the full-length huntingtin and HTT1a tra...

Descripción completa

Detalles Bibliográficos
Autores principales:	Smith, Edward J, Sathasivam, Kirupa, Landles, Christian, Osborne, Georgina F, Mason, Michael A, Gomez-Paredes, Casandra, Taxy, Bridget A, Milton, Rebecca E, Ast, Anne, Schindler, Franziska, Zhang, Chuangchuang, Duan, Wenzhen, Wanker, Erich E, Bates, Gillian P
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2023
Materias:	Original Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9901570/ https://www.ncbi.nlm.nih.gov/pubmed/36756307 http://dx.doi.org/10.1093/braincomms/fcad010

Ejemplares similares

Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington’s Disease Knock-in Mice
por: Schindler, Franziska, et al.
Publicado: (2021)

Development of novel bioassays to detect soluble and aggregated Huntingtin proteins on three technology platforms
por: Landles, Christian, et al.
Publicado: (2021)

Subcellular Localization And Formation Of Huntingtin Aggregates Correlates With Symptom Onset And Progression In A Huntington’S Disease Model
por: Landles, Christian, et al.
Publicado: (2020)

Extensive Expression Analysis of Htt Transcripts in Brain Regions from the zQ175 HD Mouse Model Using a QuantiGene Multiplex Assay
por: Papadopoulou, Aikaterini S., et al.
Publicado: (2019)

Abnormal Brain Development in Huntington’ Disease Is Recapitulated in the zQ175 Knock-In Mouse Model
por: Zhang, Chuangchuang, et al.
Publicado: (2020)

Silencing Srsf6 does not modulate incomplete splicing of the huntingtin gene in Huntington’s disease models
por: Mason, Michael A., et al.
Publicado: (2020)

Alternative processing of human HTT mRNA with implications for Huntington’s disease therapeutics
por: Fienko, Sandra, et al.
Publicado: (2022)

Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington’s disease
por: Zhou, Ya, et al.
Publicado: (2021)

Characterization of Behavioral, Neuropathological, Brain Metabolic and Key Molecular Changes in zQ175 Knock-In Mouse Model of Huntington’s Disease
por: Peng, Qi, et al.
Publicado: (2016)

Enhanced GABAergic Inhibition of Cholinergic Interneurons in the zQ175(+/−) Mouse Model of Huntington's Disease
por: Lim, Sean Austin O., et al.
Publicado: (2021)

Cognitive Training at a Young Age Attenuates Deficits in the zQ175 Mouse Model of HD
por: Curtin, Paul C. P., et al.
Publicado: (2016)

Metformin treatment reduces motor and neuropsychiatric phenotypes in the zQ175 mouse model of Huntington disease
por: Sanchis, Ana, et al.
Publicado: (2019)

The Urine Metabolome of R6/2 and zQ175DN Huntington’s Disease Mouse Models
por: Speziale, Roberto, et al.
Publicado: (2023)

Genetic Deletion of Transglutaminase 2 Does Not Rescue the Phenotypic Deficits Observed in R6/2 and zQ175 Mouse Models of Huntington's Disease
por: Menalled, Liliana B., et al.
Publicado: (2014)

Comprehensive Behavioral and Molecular Characterization of a New Knock-In Mouse Model of Huntington’s Disease: zQ175
por: Menalled, Liliana B., et al.
Publicado: (2012)

HDAC4 Reduction: A Novel Therapeutic Strategy to Target Cytoplasmic Huntingtin and Ameliorate Neurodegeneration
por: Mielcarek, Michal, et al.
Publicado: (2013)

Search for t to Zq
por: CMS Collaboration
Publicado: (2012)

The heat shock response, determined by QuantiGene multiplex, is impaired in HD mouse models and not caused by HSF1 reduction
por: Gomez-Paredes, Casandra, et al.
Publicado: (2021)

Optimising Golgi–Cox staining for use with perfusion-fixed brain tissue validated in the zQ175 mouse model of Huntington's disease
por: Bayram-Weston, Zubeyde, et al.
Publicado: (2016)

Cell-Type Specific Regulation of Cholesterogenesis by CYP46A1 Re-Expression in zQ175 HD Mouse Striatum
por: Pinchaud, Katleen, et al.
Publicado: (2023)

Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington’s disease
por: Landles, Christian, et al.
Publicado: (2012)

Characterization of Neurophysiological and Behavioral Changes, MRI Brain Volumetry and 1H MRS in zQ175 Knock-In Mouse Model of Huntington's Disease
por: Heikkinen, Taneli, et al.
Publicado: (2012)

Characterization of HTT Inclusion Size, Location, and Timing in the zQ175 Mouse Model of Huntington´s Disease: An In Vivo High-Content Imaging Study
por: Carty, Nikisha, et al.
Publicado: (2015)

Metabotropic Glutamate Receptor 5 Antagonism Reduces Pathology and Differentially Improves Symptoms in Male and Female Heterozygous zQ175 Huntington’s Mice
por: Li, Si Han, et al.
Publicado: (2022)

Longitudinal investigation of changes in resting-state co-activation patterns and their predictive ability in the zQ175 DN mouse model of Huntington’s disease
por: Adhikari, Mohit H., et al.
Publicado: (2023)

Small Molecule Pytren-4QMn Metal Complex Slows down Huntington’s Disease Progression in Male zQ175 Transgenic Mice
por: Merino, Marián, et al.
Publicado: (2023)

Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1
por: van der Bent, M Leontien, et al.
Publicado: (2022)

Phenotype onset in Huntington’s disease knock‐in mice is correlated with the incomplete splicing of the mutant huntingtin gene
por: Franich, Nicholas R., et al.
Publicado: (2019)

Modulation of mTOR and CREB pathways following mGluR5 blockade contribute to improved Huntington’s pathology in zQ175 mice
por: Abd-Elrahman, Khaled S., et al.
Publicado: (2019)

Huntingtin exon 1 deletion does not alter the subcellular distribution of huntingtin and gene transcription in mice
por: Zhao, Xianxian, et al.
Publicado: (2022)

A mixed fixed ratio/progressive ratio procedure reveals an apathy phenotype in the BAC HD and the z_Q175 KI mouse models of Huntington’s disease
por: Oakeshott, Stephen, et al.
Publicado: (2012)

Truncation of mutant huntingtin in knock-in mice demonstrates exon1 huntingtin is a key pathogenic form
por: Yang, Huiming, et al.
Publicado: (2020)

Glial Cell Lineage Expression of Mutant Ataxin-1 and Huntingtin Induces Developmental and Late-Onset Neuronal Pathologies in Drosophila Models
por: Tamura, Takuya, et al.
Publicado: (2009)

Bifunctional Anti-Huntingtin Proteasome-Directed Intrabodies Mediate Efficient Degradation of Mutant Huntingtin Exon 1 Protein Fragments
por: Butler, David C., et al.
Publicado: (2011)

Author Correction: Truncation of mutant huntingtin in knock-in mice demonstrates exon1 huntingtin is a key pathogenic form
por: Yang, Huiming, et al.
Publicado: (2020)

Polyglutamine Induced Misfolding of Huntingtin Exon1 is Modulated by the Flanking Sequences
por: Lakhani, Vinal V., et al.
Publicado: (2010)

Aggregation Behavior of Chemically Synthesized, Full-Length Huntingtin Exon1
por: Sahoo, Bankanidhi, et al.
Publicado: (2014)

Perturbation with Intrabodies Reveals That Calpain Cleavage Is Required for Degradation of Huntingtin Exon 1
por: Southwell, Amber L., et al.
Publicado: (2011)

Discovery of Novel Isoforms of Huntingtin Reveals a New Hominid-Specific Exon
por: Ruzo, Albert, et al.
Publicado: (2015)

Study of ATLAS Sensitivity to FCNC TOP Quark Decay $t \to Zq$
por: Chikovani, Leila, et al.
Publicado: (2000)

Cannot write session to /tmp/vufind_sessions/sess_19f2kopnn9duhv9pn2llju2afl