Cargando…

Fibril polymorphism affects immobilized non-amyloid flanking domains of huntingtin exon1 rather than its polyglutamine core

Polyglutamine expansion in the huntingtin protein is the primary genetic cause of Huntington's disease (HD). Fragments coinciding with mutant huntingtin exon1 aggregate in vivo and induce HD-like pathology in mouse models. The resulting aggregates can have different structures that affect their...

Descripción completa

Detalles Bibliográficos
Autores principales:	Lin, Hsiang-Kai, Boatz, Jennifer C., Krabbendam, Inge E., Kodali, Ravindra, Hou, Zhipeng, Wetzel, Ronald, Dolga, Amalia M., Poirier, Michelle A., van der Wel, Patrick C. A.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group 2017
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5458082/ https://www.ncbi.nlm.nih.gov/pubmed/28537272 http://dx.doi.org/10.1038/ncomms15462

Ejemplares similares

Polyglutamine Amyloid Core Boundaries and Flanking Domain Dynamics in Huntingtin Fragment Fibrils Determined by Solid-State Nuclear Magnetic Resonance
por: Hoop, Cody L., et al.
Publicado: (2014)

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

Polyglutamine disruption of the huntingtin exon1 N-terminus triggers a complex aggregation mechanism
por: Thakur, Ashwani K., et al.
Publicado: (2009)

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

Folding Landscape of Mutant Huntingtin Exon1: Diffusible Multimers, Oligomers and Fibrils, and No Detectable Monomer
por: Sahoo, Bankanidhi, et al.
Publicado: (2016)

Huntingtin structure is orchestrated by HAP40 and shows a polyglutamine expansion-specific interaction with exon 1
por: Harding, Rachel J., et al.
Publicado: (2021)

Selective observation of semi-rigid non-core residues in dynamically complex mutant huntingtin protein fibrils
por: Matlahov, Irina, et al.
Publicado: (2022)

Monomeric Huntingtin Exon 1 Has Similar Overall Structural Features for Wild-Type and Pathological Polyglutamine Lengths
por: Warner, John B., et al.
Publicado: (2017)

Membrane Interactions Accelerate the Self-Aggregation of Huntingtin Exon 1 Fragments in a Polyglutamine Length-Dependent Manner
por: Marquette, Arnaud, et al.
Publicado: (2021)

Development of mAb-based polyglutamine-dependent and polyglutamine length-independent huntingtin quantification assays with cross-site validation
por: Fischer, David F., et al.
Publicado: (2022)

Quantification Assays for Total and Polyglutamine-Expanded Huntingtin Proteins
por: Macdonald, Douglas, et al.
Publicado: (2014)

Critical nucleus size for disease-related polyglutamine aggregation is repeat length dependent
por: Kar, Karunakar, et al.
Publicado: (2011)

Pathogenic polyglutamine expansion length correlates with polarity of the flanking sequences
por: Kim, Meewhi
Publicado: (2014)

Polyglutamine Tract Expansion Increases S-Nitrosylation of Huntingtin and Ataxin-1
por: Ni, Chun-Lun, et al.
Publicado: (2016)

Conformational switch of polyglutamine-expanded huntingtin into benign aggregates leads to neuroprotective effect
por: Sun, Chia-Sui, et al.
Publicado: (2015)

Deletion of the Huntingtin Polyglutamine Stretch Enhances Neuronal Autophagy and Longevity in Mice
por: Zheng, Shuqiu, et al.
Publicado: (2010)

Polyglutamine expansion affects huntingtin conformation in multiple Huntington’s disease models
por: Daldin, Manuel, et al.
Publicado: (2017)

Polyglutamine Expansion in Huntingtin and Mechanism of DNA Damage Repair Defects in Huntington’s Disease
por: Pradhan, Subrata, et al.
Publicado: (2022)

Integrative determination of the atomic structure of mutant huntingtin exon 1 fibrils from Huntington’s disease
por: Helabad, Mahdi Bagherpoor, et al.
Publicado: (2023)

Modifiers of mutant huntingtin aggregation: functional conservation of C. elegans-modifiers of polyglutamine aggregation
por: Teuling, Eva, et al.
Publicado: (2011)

Impaired Heat Shock Response in Cells Expressing Full-Length Polyglutamine-Expanded Huntingtin
por: Chafekar, Sidhartha M., et al.
Publicado: (2012)

Purification of full-length recombinant human huntingtin proteins with allelic series of polyglutamine lengths
por: Kim, Hyeongju, et al.
Publicado: (2021)

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)

Unbiased Gene Expression Analysis Implicates the huntingtin Polyglutamine Tract in Extra-mitochondrial Energy Metabolism
por: Lee, Jong-Min, et al.
Publicado: (2007)

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function
por: Vijayvargia, Ravi, et al.
Publicado: (2016)

Polyglutamine- and Temperature-Dependent Conformational Rigidity in Mutant Huntingtin Revealed by Immunoassays and Circular Dichroism Spectroscopy
por: Fodale, Valentina, et al.
Publicado: (2014)

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

α-Synuclein and huntingtin exon 1 amyloid fibrils bind laterally to the cellular membrane
por: Monsellier, Elodie, et al.
Publicado: (2016)

Polyglutamine-Rich Suppressors of Huntingtin Toxicity Act Upstream of Hsp70 and Sti1 in Spatial Quality Control of Amyloid-Like Proteins
por: Wolfe, Katie J., et al.
Publicado: (2014)

Correction: Polyglutamine- and Temperature-Dependent Conformational Rigidity in Mutant Huntingtin Revealed by Immunoassays and Circular Dichroism Spectroscopy
Publicado: (2015)

Quantifying Huntingtin Protein in Human Cerebrospinal Fluid Using a Novel Polyglutamine Length-Independent Assay
por: Fodale, Valentina, 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)

Control of the structural landscape and neuronal proteotoxicity of mutant Huntingtin by domains flanking the polyQ tract
por: Shen, Koning, et al.
Publicado: (2016)

Huntingtin toxicity in yeast model depends on polyglutamine aggregation mediated by a prion-like protein Rnq1
por: Meriin, Anatoli B., et al.
Publicado: (2002)

Reversal of a full-length mutant huntingtin neuronal cell phenotype by chemical inhibitors of polyglutamine-mediated aggregation
por: Wang, Jin, et al.
Publicado: (2005)

Conformational studies of pathogenic expanded polyglutamine protein deposits from Huntington’s disease
por: Matlahov, Irina, et al.
Publicado: (2019)

Correlation of Inter-Locus Polyglutamine Toxicity with CAG•CTG Triplet Repeat Expandability and Flanking Genomic DNA GC Content
por: Nestor, Colm E., et al.
Publicado: (2011)

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)

Structural Fingerprinting of Protein Aggregates by Dynamic Nuclear Polarization-Enhanced Solid-State NMR at Natural Isotopic Abundance
por: Smith, Adam N., et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_99kbqkjqe5cbisve91v8lm5ujl