Cargando…

Promoters Are Differentially Sensitive to N-Terminal Mutant Huntingtin-Mediated Transcriptional Repression

Huntington’s disease (HD) is a neurodegenerative disorder caused by the inheritance of one mutant copy of the huntingtin gene. Mutant huntingtin protein (mHtt) contains an expanded polyglutamine repeat region near the N-terminus. Cleavage of mHtt releases an N-terminal fragment (N-mHtt) which accumu...

Descripción completa

Detalles Bibliográficos
Autores principales:	Hogel, Matthew, Laprairie, Robert B., Denovan-Wright, Eileen M.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Public Library of Science 2012
Materias:	Research Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3399790/ https://www.ncbi.nlm.nih.gov/pubmed/22815947 http://dx.doi.org/10.1371/journal.pone.0041152

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)

Modulation of Mutant Huntingtin N-Terminal Cleavage and Its Effect on Aggregation and Cell Death
por: Juenemann, Katrin, et al.
Publicado: (2010)

Subfunctionalization of peroxisome proliferator response elements accounts for retention of duplicated fabp1 genes in zebrafish
por: Laprairie, Robert B., et al.
Publicado: (2016)

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)

Mutant Huntingtin stalls ribosomes and represses protein synthesis in a cellular model of Huntington disease
por: Eshraghi, Mehdi, et al.
Publicado: (2021)

Components of the endocannabinoid and dopamine systems are dysregulated in Huntington's disease: analysis of publicly available microarray datasets
por: Laprairie, Robert B, et al.
Publicado: (2015)

Huntingtin facilitates polycomb repressive complex 2
por: Seong, Ihn Sik, et al.
Publicado: (2010)

Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II
por: Benchoua, Alexandra, et al.
Publicado: (2008)

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)

Wild-type but not mutant huntingtin modulates the transcriptional activity of liver X receptors
por: Futter, M, et al.
Publicado: (2009)

Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription
por: Gao, Rui, et al.
Publicado: (2019)

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

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

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)

Sirt1 mediates neuroprotection from mutant huntingtin by activation of TORC1 and CREB transcriptional pathway
por: Cohen, Dena E., et al.
Publicado: (2011)

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)

Transcriptional Regulation of the Huntingtin Gene
por: Thomson, Sarah B., et al.
Publicado: (2018)

Bifunctional Anti-Huntingtin Proteasome-Directed Intrabodies Mediate Efficient Degradation of Mutant Huntingtin Exon 1 Protein Fragments
por: Butler, David C., et al.
Publicado: (2011)

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)

Deimmunization for gene therapy: host matching of synthetic zinc finger constructs enables long-term mutant Huntingtin repression in mice
por: Agustín-Pavón, Carmen, et al.
Publicado: (2016)

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

Differential transcriptional modulation of duplicated fatty acid-binding protein genes by dietary fatty acids in zebrafish (Danio rerio): evidence for subfunctionalization or neofunctionalization of duplicated genes
por: Karanth, Santhosh, et al.
Publicado: (2009)

A Specific Mini‐Intrabody Mediates Lysosome Degradation of Mutant Huntingtin
por: Li, Caijuan, et al.
Publicado: (2023)

Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels
por: Paul, Bindu D., 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)

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease
por: Ehrnhoefer, Dagmar E., et al.
Publicado: (2018)

The Proteasome Activators Blm10/PA200 Enhance the Proteasomal Degradation of N-Terminal Huntingtin
por: Aladdin, Azzam, et al.
Publicado: (2020)

Targeting N-Terminal Huntingtin with a Dual-sgRNA Strategy by CRISPR/Cas9
por: Wu, Junjiao, et al.
Publicado: (2019)

The Wnt Receptor Ryk Reduces Neuronal and Cell Survival Capacity by Repressing FOXO Activity During the Early Phases of Mutant Huntingtin Pathogenicity
por: Tourette, Cendrine, et al.
Publicado: (2014)

Mutant huntingtin fragment selectively suppresses Brn-2 POU domain transcription factor to mediate hypothalamic cell dysfunction
por: Yamanaka, Tomoyuki, et al.
Publicado: (2010)

Mutant CAG repeats of Huntingtin transcript fold into hairpins, form nuclear foci and are targets for RNA interference
por: de Mezer, Mateusz, et al.
Publicado: (2011)

Cannot write session to /tmp/vufind_sessions/sess_al2hs4uklmbb9traje7n8fqken