Cargando…

Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1

Huntington’s disease is a devastating heritable neurodegenerative disorder that is caused by the presence of a trinucleotide CAG repeat expansion in the Huntingtin gene, leading to a polyglutamine tract in the protein. Various mechanisms lead to the production of N-terminal Huntingtin protein fragme...

Descripción completa

Detalles Bibliográficos
Autores principales:	van der Bent, M Leontien, Evers, Melvin M, Vallès, Astrid
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Dove 2022
Materias:	Review
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9532260/ https://www.ncbi.nlm.nih.gov/pubmed/36213816 http://dx.doi.org/10.2147/BTT.S270657

Ejemplares similares

N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy
por: Liu, Xudong, et al.
Publicado: (2016)

AAV5-miHTT Gene Therapy Demonstrates Sustained Huntingtin Lowering and Functional Improvement in Huntington Disease Mouse Models
por: Spronck, Elisabeth A., et al.
Publicado: (2019)

Making (anti-) sense out of huntingtin levels in Huntington disease
por: Evers, Melvin M, et al.
Publicado: (2015)

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

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)

Huntingtin Aggregates in the Olfactory Bulb in Huntington’s Disease
por: Highet, Blake, et al.
Publicado: (2020)

Huntingtin exon 1 deletion does not alter the subcellular distribution of huntingtin and gene transcription in mice
por: Zhao, Xianxian, 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)

AAV5-miHTT Gene Therapy Demonstrates Broad Distribution and Strong Human Mutant Huntingtin Lowering in a Huntington’s Disease Minipig Model
por: Evers, Melvin M., et al.
Publicado: (2018)

Effect of post-mortem delay on N-terminal huntingtin protein fragments in human control and Huntington disease brain lysates
por: Schut, Menno H., et al.
Publicado: (2017)

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

Huntington’s Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy
por: Sari, Youssef
Publicado: (2011)

Selection and characterization of llama single domain antibodies against N-terminal huntingtin
por: Schut, Menno H., et al.
Publicado: (2014)

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)

Salivary levels of total huntingtin are elevated in Huntington’s disease patients
por: Corey-Bloom, Jody, et al.
Publicado: (2018)

Recent Trends in Detection of Huntingtin and Preclinical Models of Huntington's Disease
por: Mantha, Neelima, et al.
Publicado: (2014)

Ultrasensitive measurement of huntingtin protein in cerebrospinal fluid demonstrates increase with Huntington disease stage and decrease following brain huntingtin suppression
por: Southwell, Amber L., et al.
Publicado: (2015)

The distribution and density of Huntingtin inclusions across the Huntington disease neocortex: regional correlations with Huntingtin repeat expansion independent of pathologic grade
por: Hickman, Richard A., et al.
Publicado: (2022)

A role of mitochondrial complex II defects in genetic models of Huntington's disease expressing N-terminal fragments of mutant huntingtin
por: Damiano, Maria, et al.
Publicado: (2013)

Salivary Huntingtin protein is uniquely associated with clinical features of Huntington’s disease
por: Parkin, Georgia M., et al.
Publicado: (2023)

Rac1 Activity Is Modulated by Huntingtin and Dysregulated in Models of Huntington’s Disease
por: Tousley, Adelaide, et al.
Publicado: (2019)

Huntingtin and the Synapse
por: Barron, Jessica C., et al.
Publicado: (2021)

Soluble mutant huntingtin drives early human pathogenesis in Huntington’s disease
por: Miguez, Andrés, et al.
Publicado: (2023)

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

Embryonic Mutant Huntingtin Aggregate Formation in Mouse Models of Huntington’s Disease
por: Osmand, Alexander P., et al.
Publicado: (2016)

A Fresh Look at Huntingtin mRNA Processing in Huntington’s Disease
por: Romo, Lindsay, et al.
Publicado: (2018)

Huntington’s Disease Protein Huntingtin Associates with its own mRNA
por: Culver, Brady P., et al.
Publicado: (2016)

Analysis of mutant and total huntingtin expression in Huntington’s disease murine models
por: Fodale, Valentina, et al.
Publicado: (2020)

Huntingtin Ubiquitination Mechanisms and Novel Possible Therapies to Decrease the Toxic Effects of Mutated Huntingtin
por: Fiorillo, Annarita, et al.
Publicado: (2021)

Promoters Are Differentially Sensitive to N-Terminal Mutant Huntingtin-Mediated Transcriptional Repression
por: Hogel, Matthew, et al.
Publicado: (2012)

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

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

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)

A pathogenic proteolysis–resistant huntingtin isoform induced by an antisense oligonucleotide maintains huntingtin function
por: Kim, Hyeongju, et al.
Publicado: (2022)

A Huntingtin Peptide Inhibits PolyQ-Huntingtin Associated Defects
por: Arribat, Yoan, et al.
Publicado: (2013)

The evolution of the huntingtin-associated protein 40 (HAP40) in conjunction with huntingtin
por: Seefelder, Manuel, et al.
Publicado: (2020)

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

AAV5-miHTT Lowers Huntingtin mRNA and Protein without Off-Target Effects in Patient-Derived Neuronal Cultures and Astrocytes
por: Keskin, Sonay, et al.
Publicado: (2019)

Cannot write session to /tmp/vufind_sessions/sess_32cretqpuhocm89pjivj8cv2gm