Cargando…

Analysis of mutant and total huntingtin expression in Huntington’s disease murine models

Huntington’s disease (HD) is a monogenetic neurodegenerative disorder that is caused by the expansion of a polyglutamine region within the huntingtin (HTT) protein, but there is still an incomplete understanding of the molecular mechanisms that drive pathology. Expression of the mutant form of HTT i...

Descripción completa

Detalles Bibliográficos
Autores principales:	Fodale, Valentina, Pintauro, Roberta, Daldin, Manuel, Altobelli, Roberta, Spiezia, Maria Carolina, Bisbocci, Monica, Macdonald, Douglas, Bresciani, Alberto
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Nature Publishing Group UK 2020
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7746729/ https://www.ncbi.nlm.nih.gov/pubmed/33335120 http://dx.doi.org/10.1038/s41598-020-78790-5

Ejemplares similares

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

Validation of Ultrasensitive Mutant Huntingtin Detection in Human Cerebrospinal Fluid by Single Molecule Counting Immunoassay
por: Fodale, Valentina, et al.
Publicado: (2017)

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

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

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

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 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)

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

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

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

Quantifying autophagy using novel LC3B and p62 TR-FRET assays
por: Bresciani, Alberto, et al.
Publicado: (2018)

Mutant Huntingtin Does Not Affect the Intrinsic Phenotype of Human Huntington’s Disease T Lymphocytes
por: Miller, James R. C., et al.
Publicado: (2015)

Genistein induces degradation of mutant huntingtin in fibroblasts from Huntington’s disease patients
por: Pierzynowska, Karolina, et al.
Publicado: (2019)

Neuronal Targets of Mutant Huntingtin Genetic Reduction to Ameliorate Huntington’s Disease Pathogenesis in Mice
por: Wang, Nan, et al.
Publicado: (2014)

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

Mutant Huntingtin Affects Diabetes and Alzheimer’s Markers in Human and Cell Models of Huntington’s Disease
por: Chaves, Gepoliano, et al.
Publicado: (2019)

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

Esculetin Provides Neuroprotection against Mutant Huntingtin-Induced Toxicity in Huntington’s Disease Models
por: Pruccoli, Letizia, et al.
Publicado: (2021)

Effects of mutant huntingtin inactivation on Huntington disease‐related behaviours in the BACHD mouse model
por: Cheong, Rachel Y., et al.
Publicado: (2021)

Suppression of MAPK11 or HIPK3 reduces mutant Huntingtin levels in Huntington's disease models
por: Yu, Meng, et al.
Publicado: (2017)

Mutant huntingtin induces iron overload via up-regulating IRP1 in Huntington’s disease
por: Niu, Li, et al.
Publicado: (2018)

TBK1 phosphorylates mutant Huntingtin and suppresses its aggregation and toxicity in Huntington's disease models
por: Hegde, Ramanath Narayana, et al.
Publicado: (2020)

Osmolytes dynamically regulate mutant Huntingtin aggregation and CREB function in Huntington’s disease cell models
por: Aravindan, Shreyaas, et al.
Publicado: (2020)

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)

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

Mutant Huntingtin Derails Cysteine Metabolism in Huntington’s Disease at Both Transcriptional and Post-Translational Levels
por: Paul, Bindu D., et al.
Publicado: (2022)

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

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

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

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

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)

Inducible mutant huntingtin expression in HN10 cells reproduces Huntington's disease-like neuronal dysfunction
por: Weiss, Andreas, et al.
Publicado: (2009)

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)

Striatal Mutant Huntingtin Protein Levels Decline with Age in Homozygous Huntington’s Disease Knock-In Mouse Models
por: Franich, Nicholas R., et al.
Publicado: (2018)

Peripheral Expression of Mutant Huntingtin is a Critical Determinant of Weight Loss and Metabolic Disturbances in Huntington’s Disease
por: Lakra, Priya, et al.
Publicado: (2019)

CRISPR-Cas9 Mediated Gene-Silencing of the Mutant Huntingtin Gene in an In Vitro Model of Huntington’s Disease
por: Kolli, Nivya, et al.
Publicado: (2017)

Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington’s disease
por: Zhou, Ya, et al.
Publicado: (2021)

Targeted Genetic Reduction of Mutant Huntingtin Lessens Cardiac Pathology in the BACHD Mouse Model of Huntington's Disease
por: Park, Saemi, et al.
Publicado: (2021)

Discovery of an autophagy inducer J3 to lower mutant huntingtin and alleviate Huntington’s disease-related phenotype
por: Long, Jiahui, et al.
Publicado: (2022)

Cannot write session to /tmp/vufind_sessions/sess_m08fj47n1umrilt5ud1h8vml1g