Cargando…

Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington’s Disease Knock-In Mouse Models

BACKGROUND: Huntington’s disease (HD) is a progressive neurodegenerative disorder associated with aging, caused by an expanded polyglutamine (polyQ) repeat within the Huntingtin (HTT) protein. In HD, degeneration of the striatum and atrophy of the cortex are observed while cerebellum is less affecte...

Descripción completa

Detalles Bibliográficos
Autores principales:	Franich, Nicholas R., Basso, Manuela, André, Emily A., Ochaba, Joseph, Kumar, Amit, Thein, Soe, Fote, Gianna, Kachemov, Marketta, Lau, Alice L., Yeung, Sylvia Y., Osmand, Alexander, Zeitlin, Scott O., Ratan, Rajiv R., Thompson, Leslie M., Steffan, Joan S.
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	IOS Press 2018
Materias:	Research Report
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6002862/ https://www.ncbi.nlm.nih.gov/pubmed/29843246 http://dx.doi.org/10.3233/JHD-170274

Ejemplares similares

IKKβ slows Huntington’s disease progression in R6/1 mice
por: Ochaba, Joseph, et al.
Publicado: (2019)

Phenotype onset in Huntington’s disease knock‐in mice is correlated with the incomplete splicing of the mutant huntingtin gene
por: Franich, Nicholas R., et al.
Publicado: (2019)

Diminished LC3-Associated Phagocytosis by Huntington’s Disease Striatal Astrocytes
por: Wakida, Nicole M., et al.
Publicado: (2022)

PIAS1 modulates striatal transcription, DNA damage repair, and SUMOylation with relevance to Huntington’s disease
por: Morozko, Eva L., et al.
Publicado: (2021)

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

Msh2 Acts in Medium-Spiny Striatal Neurons as an Enhancer of CAG Instability and Mutant Huntingtin Phenotypes in Huntington’s Disease Knock-In Mice
por: Kovalenko, Marina, et al.
Publicado: (2012)

Cortical Axonal Secretion of BDNF in the Striatum Is Disrupted in the Mutant-huntingtin Knock-in Mouse Model of Huntington's Disease
por: Park, Hyungju
Publicado: (2018)

Improvement of neuropathology and transcriptional deficits in CAG 140 knock-in mice supports a beneficial effect of dietary curcumin in Huntington's disease
por: Hickey, Miriam A, et al.
Publicado: (2012)

Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HdhQ150 knock-in mouse model of Huntington’s disease
por: Landles, Christian, et al.
Publicado: (2012)

Loss of the thyroid hormone-binding protein Crym renders striatal neurons more vulnerable to mutant huntingtin in Huntington's disease
por: Francelle, Laetitia, et al.
Publicado: (2015)

Transglutaminase 6 Is Colocalized and Interacts with Mutant Huntingtin in Huntington Disease Rodent Animal Models
por: Schulze-Krebs, Anja, et al.
Publicado: (2021)

Small, Seeding-Competent Huntingtin Fibrils Are Prominent Aggregate Species in Brains of zQ175 Huntington’s Disease Knock-in Mice
por: Schindler, Franziska, et al.
Publicado: (2021)

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)

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

N-terminal Huntingtin Knock-In Mice: Implications of Removing the N-terminal Region of Huntingtin for Therapy
por: Liu, Xudong, 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)

Wild-type huntingtin ameliorates striatal neuronal atrophy but does not prevent other abnormalities in the YAC128 mouse model of Huntington disease
por: Van Raamsdonk, Jeremy M, et al.
Publicado: (2006)

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

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)

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)

Huntington’s Disease and Striatal Signaling
por: Roze, Emmanuel, et al.
Publicado: (2011)

Is Huntingtin Dispensable in the Adult Brain?
por: Liu, Jeh-Ping, et al.
Publicado: (2017)

Generation and Characterization of Knock-in Mouse Models Expressing Versions of Huntingtin with Either an N17 or a Combined PolyQ and Proline-Rich Region Deletion
por: André, Emily A., et al.
Publicado: (2017)

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

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

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)

Cortical and Striatal Circuits in Huntington’s Disease
por: Blumenstock, Sonja, et al.
Publicado: (2020)

Striatal network modeling in Huntington’s Disease
por: Ponzi, Adam, et al.
Publicado: (2020)

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)

A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity
por: Yao, Yuwei, et al.
Publicado: (2015)

The striatal kinase DCLK3 produces neuroprotection against mutant huntingtin
por: Galvan, Laurie, et al.
Publicado: (2018)

Altered exocytosis of inhibitory synaptic vesicles at single presynaptic terminals of cultured striatal neurons in a knock-in mouse model of Huntington’s disease
por: Xu, Chen, et al.
Publicado: (2023)

Similar striatal gene expression profiles in the striatum of the YAC128 and HdhQ150 mouse models of Huntington’s disease are not reflected in mutant Huntingtin inclusion prevalence
por: Bayram-Weston, Zubeyde, et al.
Publicado: (2015)

Benefits of global mutant huntingtin lowering diminish over time in a Huntington’s disease mouse model
por: Marchionini, Deanna M., et al.
Publicado: (2022)

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

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

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

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

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

Cannot write session to /tmp/vufind_sessions/sess_ecb7buvf0vcb62fthrqi4lgvjo