Cargando…

N17 Modifies Mutant Huntingtin Nuclear Pathogenesis and Severity of Disease in HD BAC Transgenic Mice

The nucleus is a critical subcellular compartment for the pathogenesis of polyglutamine disorders, including Huntington’s disease (HD). Recent studies suggest the first 17-amino-acid domain (N17) of mutant Huntingtin (mHTT) mediates its nuclear exclusion in cultured cells. Here, we test whether N17...

Descripción completa

Detalles Bibliográficos
Autores principales:	Gu, Xiaofeng, Cantle, Jeffrey P., Greiner, Erin R., Lee, C.Y. Daniel, Barth, Albert M., Gao, Fuying, Park, Chang Sin, Zhang, Zhiqiang, Sandoval-Miller, Susana, Zhang, Richard L., Diamond, Marc, Mody, Istvan, Coppola, Giovanni, Yang, X. William
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	2015
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4386927/ https://www.ncbi.nlm.nih.gov/pubmed/25661181 http://dx.doi.org/10.1016/j.neuron.2015.01.008

Ejemplares similares

Integrated genomics and proteomics to define huntingtin CAG length-dependent networks in HD Mice
por: Langfelder, Peter, et al.
Publicado: (2016)

Neuronal Targets of Mutant Huntingtin Genetic Reduction to Ameliorate Huntington’s Disease Pathogenesis in Mice
por: Wang, Nan, et al.
Publicado: (2014)

Increased Body Weight of the BAC HD Transgenic Mouse Model of Huntington’s Disease Accounts for Some but Not All of the Observed HD-like Motor Deficits
por: Kudwa, Andrea E., et al.
Publicado: (2013)

Rhes depletion promotes striatal accumulation and aggregation of mutant huntingtin in a presymptomatic HD mouse model
por: Pan, Yongcheng, et al.
Publicado: (2023)

HD CAGnome: A Search Tool for Huntingtin CAG Repeat Length-Correlated Genes
por: Galkina, Ekaterina I., et al.
Publicado: (2014)

Generation of BAC Transgenic Epithelial Organoids
por: Schwank, Gerald, et al.
Publicado: (2013)

The HSPB1-p62/SQSTM1 functional complex regulates the unconventional secretion and transcellular spreading of the HD-associated mutant huntingtin protein
por: Bonavita, R, et al.
Publicado: (2023)

Premature death and neurologic abnormalities in transgenic mice expressing a mutant huntingtin exon-2 fragment
por: Tebbenkamp, Andrew T.N., et al.
Publicado: (2011)

Neuropathology of transgenic HD animal models
por: Li, Xiaojiang, et al.
Publicado: (2012)

pS421 huntingtin modulates mitochondrial phenotypes and confers neuroprotection in an HD hiPSC model
por: Xu, Xiaohong, et al.
Publicado: (2020)

MicroRNA signatures of endogenous Huntingtin CAG repeat expansion in mice
por: Langfelder, Peter, et al.
Publicado: (2018)

Normal Aging Modulates the Neurotoxicity of Mutant Huntingtin
por: Diguet, Elsa, et al.
Publicado: (2009)

Mutant huntingtin in the hypothalamus causes metabolic dysfunction
Publicado: (2011)

Unconventional Secretion and Intercellular Transfer of Mutant Huntingtin
por: Tang, Bor Luen
Publicado: (2018)

Cancer: From Wild-Type to Mutant Huntingtin
por: Thion, Morgane Sonia, et al.
Publicado: (2018)

Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity
por: Luo, Shouqing, et al.
Publicado: (2005)

Phosphorylation of huntingtin at residue T3 is decreased in Huntington’s disease and modulates mutant huntingtin protein conformation
por: Cariulo, Cristina, et al.
Publicado: (2017)

Quantifying mutant huntingtin protein in human cerebrospinal fluid to support the development of huntingtin-lowering therapies
por: Vauleon, Stephanie, et al.
Publicado: (2023)

Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdh(Q111) Model of HD
por: Bowles, Kathryn R., et al.
Publicado: (2015)

The expanded CAG repeat in the huntingtin gene as target for therapeutic RNA modulation throughout the HD mouse brain
por: Datson, Nicole A., et al.
Publicado: (2017)

Huntingtin Co-Isolates with Small Extracellular Vesicles from Blood Plasma of TgHD and KI-HD Pig Models of Huntington’s Disease and Human Blood Plasma
por: Ananbeh, Hanadi, et al.
Publicado: (2022)

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

GRASP55 regulates the unconventional secretion and aggregation of mutant huntingtin
por: Ahat, Erpan, et al.
Publicado: (2022)

Visualization of cell-to-cell transmission of mutant huntingtin oligomers
por: Herrera, Federico, et al.
Publicado: (2011)

Dynamic recruitment of ubiquitin to mutant huntingtin inclusion bodies
por: Juenemann, Katrin, et al.
Publicado: (2018)

Characterization of axonal transport defects in Drosophila Huntingtin mutants
por: Weiss, Kurt R., et al.
Publicado: (2016)

Random Lasing Detection of Mutant Huntingtin Expression in Cells
por: de Armas-Rillo, Sergio, et al.
Publicado: (2021)

The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins
por: Folger, Austin, et al.
Publicado: (2021)

Correction: Huntingtin Subcellular Localisation Is Regulated by Kinase Signalling Activity in the StHdh(Q111) Model of HD
por: Bowles, Kathryn R., et al.
Publicado: (2016)

Efficient Production of Fluorescent Transgenic Rats using the piggyBac Transposon
por: Li, Tianda, et al.
Publicado: (2016)

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)

Tissue Transglutaminase Does Not Contribute to the Formation of Mutant Huntingtin Aggregates
por: Chun, Wanjoo, et al.
Publicado: (2001)

Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity
por: Shin, Ji-Yeon, et al.
Publicado: (2005)

Mutant huntingtin impairs Ku70-mediated DNA repair
por: Enokido, Yasushi, et al.
Publicado: (2010)

Phosphorylation of Mutant Huntingtin at Serine 116 Modulates Neuronal Toxicity
por: Watkin, Erin E., et al.
Publicado: (2014)

Huntington's disease cerebrospinal fluid seeds aggregation of mutant huntingtin
por: Tan, Z, et al.
Publicado: (2015)

Inhibition of DNA Methyltransferases Blocks Mutant Huntingtin-Induced Neurotoxicity
por: Pan, Yanchun, et al.
Publicado: (2016)

Human-to-mouse prion-like propagation of mutant huntingtin protein
por: Jeon, Iksoo, et al.
Publicado: (2016)

Comparative Analysis of Mutant Huntingtin Binding Partners in Yeast Species
por: Zhao, Yanding, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_i2o8lgsnbpbnc5cl4r67kfudhv