Cargando…

Quantification Assays for Total and Polyglutamine-Expanded Huntingtin Proteins

The expansion of a CAG trinucleotide repeat in the huntingtin gene, which produces huntingtin protein with an expanded polyglutamine tract, is the cause of Huntington's disease (HD). Recent studies have reported that RNAi suppression of polyglutamine-expanded huntingtin (mutant HTT) in HD anima...

Descripción completa

Detalles Bibliográficos
Autores principales:	Macdonald, Douglas, Tessari, Michela A., Boogaard, Ivette, Smith, Melanie, Pulli, Kristiina, Szynol, Agnieszka, Albertus, Faywell, Lamers, Marieke B. A. C., Dijkstra, Sipke, Kordt, Daniel, Reindl, Wolfgang, Herrmann, Frank, McAllister, George, Fischer, David F., Munoz-Sanjuan, Ignacio
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2014
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4016121/ https://www.ncbi.nlm.nih.gov/pubmed/24816435 http://dx.doi.org/10.1371/journal.pone.0096854

Ejemplares similares

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)

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

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

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

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

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

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

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

Quantifying Huntingtin Protein in Human Cerebrospinal Fluid Using a Novel Polyglutamine Length-Independent Assay
por: Fodale, Valentina, et al.
Publicado: (2022)

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)

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

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

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

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

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

Cytoplasmic Ubiquitin-Specific Protease 19 (USP19) Modulates Aggregation of Polyglutamine-Expanded Ataxin-3 and Huntingtin through the HSP90 Chaperone
por: He, Wen-Tian, et al.
Publicado: (2016)

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

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

Allele-Selective Suppression of Mutant Huntingtin in Primary Human Blood Cells
por: Miller, James R. C., et al.
Publicado: (2017)

Meso scale discovery-based assays for the detection of aggregated huntingtin
por: Reindl, Wolfgang, et al.
Publicado: (2019)

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

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

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

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)

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)

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)

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

Activation of p38MAPK Contributes to Expanded Polyglutamine-Induced Cytotoxicity
por: Tsirigotis, Maria, et al.
Publicado: (2008)

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)

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

A series of N-terminal epitope tagged Hdh knock-in alleles expressing normal and mutant huntingtin: their application to understanding the effect of increasing the length of normal huntingtin’s polyglutamine stretch on CAG140 mouse model pathogenesis
por: Zheng, Shuqiu, et al.
Publicado: (2012)

Polyglutamine-expanded androgen receptor interferes with TFEB to elicit pathological autophagy defects in SBMA
por: Cortes, Constanza J., et al.
Publicado: (2014)

The polyglutamine-expanded androgen receptor has increased DNA binding and reduced transcriptional activity
por: Belikov, Sergey, et al.
Publicado: (2015)

Impaired Nuclear Export of Polyglutamine-Expanded Androgen Receptor in Spinal and Bulbar Muscular Atrophy
por: Arnold, Frederick J., et al.
Publicado: (2019)

Cognitive Changes in the Spinocerebellar Ataxias Due to Expanded Polyglutamine Tracts: A Survey of the Literature
por: Lindsay, Evelyn, et al.
Publicado: (2017)

Mutant huntingtin confers cell-autonomous phenotypes on Huntington’s disease iPSC-derived microglia
por: Stöberl, Nina, et al.
Publicado: (2023)

Autophagy and polyglutamine diseases
por: Jimenez-Sanchez, Maria, et al.
Publicado: (2012)

Polyglutamine Repeats in Viruses
por: Schein, Catherine H.
Publicado: (2018)

Intracellular Aggregation of Polypeptides with Expanded Polyglutamine Domain Is Stimulated by Stress-Activated Kinase Mekk1
por: Meriin, Anatoli B., et al.
Publicado: (2001)

Cannot write session to /tmp/vufind_sessions/sess_evdhv6uhqn1digkvmsjvjtv9tr