Cargando…

Polyglutamine disruption of the huntingtin exon1 N-terminus triggers a complex aggregation mechanism

Simple polyglutamine (polyQ) peptides aggregate in vitro via a nucleated growth pathway directly yielding amyloid-like aggregates. We show here that the 17 amino acid flanking sequence (htt(NT)) N-terminal to the polyQ in the toxic huntingtin exon1 fragment imparts onto this peptide a complex altern...

Descripción completa

Detalles Bibliográficos
Autores principales:	Thakur, Ashwani K., Jayaraman, Murali, Mishra, Rakesh, Thakur, Monika, Chellgren, Veronique M., Byeon, In-Ja, Anjum, Dalaver H., Kodali, Ravindra, Creamer, Trevor P., Conway, James F., M.Gronenborn, Angela, Wetzel, Ronald
Formato:	Texto
Lenguaje:	English
Publicado:	2009
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2706102/ https://www.ncbi.nlm.nih.gov/pubmed/19270701 http://dx.doi.org/10.1038/nsmb.1570

Ejemplares similares

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

Fibril polymorphism affects immobilized non-amyloid flanking domains of huntingtin exon1 rather than its polyglutamine core
por: Lin, Hsiang-Kai, et al.
Publicado: (2017)

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

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

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)

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)

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

Anhydrous trifluoroacetic acid pretreatment converts insoluble polyglutamine peptides to soluble monomers
por: Burra, Gunasekhar, et al.
Publicado: (2015)

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

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)

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)

Fluorescence correlation spectroscopy shows that monomeric polyglutamine molecules form collapsed structures in aqueous solutions
por: Crick, Scott L., et al.
Publicado: (2006)

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

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

HSP90 recognizes the N-terminus of huntingtin involved in regulation of huntingtin aggregation by USP19
por: He, Wen-Tian, et al.
Publicado: (2017)

An Amyloidogenic Sequence at the N-Terminus of the Androgen Receptor Impacts Polyglutamine Aggregation
por: Oppong, Emmanuel, et al.
Publicado: (2017)

Rapid α-oligomer formation mediated by the Aβ C terminus initiates an amyloid assembly pathway
por: Misra, Pinaki, et al.
Publicado: (2016)

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)

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)

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)

The Role of Post-translational Modifications on the Energy Landscape of Huntingtin N-Terminus
por: Yalinca, Havva, 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)

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)

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)

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)

In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects
por: Faideau, Mathilde, et al.
Publicado: (2010)

Beta conformation of polyglutamine track revealed by a crystal structure of Huntingtin N-terminal region with insertion of three histidine residues
por: Kim, Meewhi
Publicado: (2013)

Glycogen synthase protects neurons from cytotoxicity of mutant huntingtin by enhancing the autophagy flux
por: Rai, Anupama, et al.
Publicado: (2018)

Cannot write session to /tmp/vufind_sessions/sess_70nuifn5c47ort76ip18hm72t6