An Expanded Polyproline Domain Maintains Mutant Huntingtin Soluble in vivo and During Aging

An Expanded Polyproline Domain Maintains Mutant Huntingtin Soluble in vivo and During Aging

Huntington’s disease is a dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat, encoding for the amino acid glutamine (Q), present in the first exon of the protein huntingtin. Over the threshold of Q39 HTT exon 1 (HTTEx1) tends to misfold and aggregate into large i...

Szczegółowa specyfikacja

Opis bibliograficzny
Główni autorzy:	Maria Lucia Pigazzini, Mandy Lawrenz, Anca Margineanu, Gabriele S. Kaminski Schierle, Janine Kirstein
Format:	Artykuł
Język:	English
Wydane:	Frontiers Media S.A. 2021-10-01
Seria:	Frontiers in Molecular Neuroscience
Hasła przedmiotowe:	C. elegans huntingtin (HTT) polyQ proline aggregation fluorescence life time imaging
Dostęp online:	https://www.frontiersin.org/articles/10.3389/fnmol.2021.721749/full

Podobne zapisy

Unconventional Secretion and Intercellular Transfer of Mutant Huntingtin
od: Bor Luen Tang
Wydane: (2018-06-01)

Rapamycin reduces neuronal mutant huntingtin aggregation and ameliorates locomotor performance in Drosophila
od: Jonathan R. Roth, i wsp.
Wydane: (2023-09-01)

Evidence for the spread of human-derived mutant huntingtin protein in mice and non-human primates
od: Philippe Gosset, i wsp.
Wydane: (2020-07-01)

Midazolam Enhances Mutant Huntingtin Protein Accumulation via Impairment of Autophagic Degradation In Vitro
od: Jiqian Zhang, i wsp.
Wydane: (2018-07-01)

The Proteasome Activators Blm10/PA200 Enhance the Proteasomal Degradation of N-Terminal Huntingtin
od: Azzam Aladdin, i wsp.
Wydane: (2020-11-01)

Use of adeno-associated virus-mediated delivery of mutant huntingtin to study the spreading capacity of the protein in mice and non-human primates
od: Alexander Maxan, i wsp.
Wydane: (2020-07-01)

Haloperidol protects striatal neurons from dysfunction induced by mutated huntingtin in vivo
od: Delphine Charvin, i wsp.
Wydane: (2008-01-01)

Full-length huntingtin is palmitoylated at multiple sites and post-translationally myristoylated following caspase-cleavage
od: Fanny L. Lemarié, i wsp.
Wydane: (2023-01-01)

Huntingtin lowering impairs the maturation and synchronized synaptic activity of human cortical neuronal networks derived from induced pluripotent stem cells
od: Mathilde Louçã, i wsp.
Wydane: (2024-10-01)

Huntingtin CAG-expansion mutation results in a dominant negative effect
od: Tiago L. Laundos, i wsp.
Wydane: (2023-09-01)

Aberrant splicing of mutant huntingtin in Huntington's disease knock-in pigs
od: Huichun Tong, i wsp.
Wydane: (2023-10-01)

Treadmill exercise delays the onset of non-motor behaviors and striatal pathology in the CAG140 knock-in mouse model of Huntington's disease
od: D.P. Stefanko, i wsp.
Wydane: (2017-09-01)

A series of N-terminal epitope tagged <it>Hdh</it> 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
od: Zheng Shuqiu, i wsp.
Wydane: (2012-08-01)

PolyQ-Expansion Causes Mitochondria Fragmentation Independent of Huntingtin and Is Distinct from Traumatic Brain Injury (TBI)/Mechanical Stress-Mediated Fragmentation Which Results from Cell Death
od: Kelsey Swinter, i wsp.
Wydane: (2023-10-01)

A guide to selecting high-performing antibodies for Huntingtin (UniProt ID: P42858) for use in western blot, immunoprecipitation, and immunofluorescence [version 2; peer review: 2 approved]
od: Rachel J. Harding, i wsp.
Wydane: (2025-01-01)

A guide to selecting high-performing antibodies for Huntingtin (UniProt ID: P42858) for use in western blot, immunoprecipitation, and immunofluorescence [version 1; peer review: 2 approved]
od: Rachel J. Harding, i wsp.
Wydane: (2024-08-01)

Pridopidine protects neurons from mutant-huntingtin toxicity via the sigma-1 receptor
od: Chelsy R. Eddings, i wsp.
Wydane: (2019-09-01)

Reduction of mutant huntingtin accumulation and toxicity by lysosomal cathepsins D and B in neurons
od: Ouyang Xiaosen, i wsp.
Wydane: (2011-06-01)

Xyloketal-derived small molecules show protective effect by decreasing mutant Huntingtin protein aggregates in Caenorhabditis elegans model of Huntington’s disease
od: Zeng YX, i wsp.
Wydane: (2016-04-01)

Rescue of aberrant huntingtin palmitoylation ameliorates mutant huntingtin-induced toxicity
od: Fanny L. Lemarié, i wsp.
Wydane: (2021-10-01)

Cerebrospinal fluid mutant huntingtin is a biomarker for huntingtin lowering in the striatum of Huntington disease mice
od: Nicholas S. Caron, i wsp.
Wydane: (2022-05-01)

Rhes depletion promotes striatal accumulation and aggregation of mutant huntingtin in a presymptomatic HD mouse model
od: Yongcheng Pan, i wsp.
Wydane: (2023-08-01)

Oleuropein enhances proteasomal activity and reduces mutant huntingtin-induced cytotoxicity
od: Zih-Ning Huang, i wsp.
Wydane: (2024-09-01)

Depletion of CBP is directly linked with cellular toxicity caused by mutant huntingtin
od: Haibing Jiang, i wsp.
Wydane: (2006-09-01)

Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death
od: Paola Conforti, i wsp.
Wydane: (2013-02-01)

Discovery of an autophagy inducer J3 to lower mutant huntingtin and alleviate Huntington’s disease-related phenotype
od: Jiahui Long, i wsp.
Wydane: (2022-10-01)

Oral administration of the cannabigerol derivative VCE-003.2 promotes subventricular zone neurogenesis and protects against mutant huntingtin-induced neurodegeneration
od: José Aguareles, i wsp.
Wydane: (2019-03-01)

The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins
od: Austin Folger, i wsp.
Wydane: (2021-10-01)

Quinic acid protects against the development of Huntington’s disease in Caenorhabditis elegans model
od: Reem Hossam El Din, i wsp.
Wydane: (2024-10-01)

Tissue Transglutaminase Selectively Modifies Proteins Associated with Truncated Mutant Huntingtin in Intact Cells
od: Wanjoo Chun, i wsp.
Wydane: (2001-06-01)

Preventing mutant huntingtin proteolysis and intermittent fasting promote autophagy in models of Huntington disease
od: Dagmar E. Ehrnhoefer, i wsp.
Wydane: (2018-03-01)

Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment
od: Diana Zala, i wsp.
Wydane: (2005-12-01)

The evolution of the huntingtin-associated protein 40 (HAP40) in conjunction with huntingtin
od: Manuel Seefelder, i wsp.
Wydane: (2020-12-01)

A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity
od: Yuwei Yao, i wsp.
Wydane: (2015-03-01)

Levels of mutant huntingtin influence the phenotypic severity of Huntington disease in YAC128 mouse models
od: Rona K. Graham, i wsp.
Wydane: (2006-02-01)

Esculetin Provides Neuroprotection against Mutant Huntingtin-Induced Toxicity in Huntington’s Disease Models
od: Letizia Pruccoli, i wsp.
Wydane: (2021-10-01)

Phagocytic glia are obligatory intermediates in transmission of mutant huntingtin aggregates across neuronal synapses
od: Kirby M Donnelly, i wsp.
Wydane: (2020-05-01)

Functional Characterisation of the Circular RNA, <i>circHTT(2-6)</i>, in Huntington’s Disease
od: Laura Gantley, i wsp.
Wydane: (2023-05-01)

Metabotropic Glutamate Receptor 5 Antagonism Reduces Pathology and Differentially Improves Symptoms in Male and Female Heterozygous zQ175 Huntington’s Mice
od: Si Han Li, i wsp.
Wydane: (2022-02-01)

Huntingtin and the Synapse
od: Jessica C. Barron, i wsp.
Wydane: (2021-06-01)