Inhibition of polyglutamine aggregation by SIMILAR huntingtin N‐terminal sequences: Prospective molecules for preclinical evaluation in Huntington's disease
Sign Up to like & getrecommendations! Published in 2017 at "Peptide Science"
DOI: 10.1002/bip.23021
Abstract: The mutant huntingtin protein (mHtt) fragments with expanded polyglutamine sequence forms microscopically visible aggregates in neurons, a hallmark of Huntington's disease (HD). The aggregation process and aggregates are possible targets of therapeutic intervention in HD.… read more here.
Keywords: huntingtin; aggregation; huntington disease; preclinical evaluation ... See more keywords
Stabilization of elongated polyglutamine tracts by a helical peptide derived from N‐terminal huntingtin
Sign Up to like & getrecommendations! Published in 2020 at "IUBMB Life"
DOI: 10.1002/iub.2288
Abstract: In Huntington's disease, the length of the polyglutamine tract in the mutant protein correlates positively with the formation of aggregates and disease symptoms and severity of the disease. Some disease‐modifying factors exist. However, no organized… read more here.
Keywords: huntingtin; length; aggregation; peptide ... See more keywords
RNA Related Pathology in Huntington's Disease.
Sign Up to like & getrecommendations! Published in 2018 at "Advances in experimental medicine and biology"
DOI: 10.1007/978-3-319-71779-1_4
Abstract: This chapter summarises research investigating the expression of huntingtin sense and anti-sense transcripts, the effect of the mutation on huntingtin processing as well as the more global effect of the mutation on the coding and… read more here.
Keywords: huntingtin; repeat; disease; pathology ... See more keywords
Energy Metabolism and Mitochondrial Superoxide Anion Production in Pre-symptomatic Striatal Neurons Derived from Human-Induced Pluripotent Stem Cells Expressing Mutant Huntingtin
Sign Up to like & getrecommendations! Published in 2019 at "Molecular Neurobiology"
DOI: 10.1007/s12035-019-01734-2
Abstract: In the present study, we investigated whether mutant huntingtin (mHTT) impairs mitochondrial functions in human striatal neurons derived from induced pluripotent stem cells (iPSCs). Striatal neurons and astrocytes derived from iPSCs from unaffected individuals (Ctrl)… read more here.
Keywords: huntingtin; anion production; striatal neurons; superoxide anion ... See more keywords
Calpastatin ablation aggravates the molecular phenotype in cell and animal models of Huntington disease
Sign Up to like & getrecommendations! Published in 2018 at "Neuropharmacology"
DOI: 10.1016/j.neuropharm.2018.01.022
Abstract: ABSTRACT Deciphering the molecular pathology of Huntington disease is of particular importance, not only for a better understanding of this neurodegenerative disease, but also to identify potential therapeutic targets. The polyglutamine‐expanded disease protein huntingtin was… read more here.
Keywords: huntingtin; aggregation; cleavage; huntington disease ... See more keywords
Small-Molecule Disruptors of Mutant Huntingtin-Calmodulin Protein-Protein Interaction Attenuate Deleterious Effects of Mutant Huntingtin.
Sign Up to like & getrecommendations! Published in 2022 at "ACS chemical neuroscience"
DOI: 10.1021/acschemneuro.2c00305
Abstract: Huntington's disease is a progressive and lethal neurodegenerative disease caused by an increased CAG repeat mutation in exon 1 of the huntingtin gene (mutant huntingtin). Current drug treatments provide only limited symptomatic relief without impacting… read more here.
Keywords: huntington disease; huntingtin; mutant huntingtin; huntingtin calmodulin ... See more keywords
The evolutionary history of the polyQ tract in huntingtin sheds light on its functional pro-neural activities
Sign Up to like & getrecommendations! Published in 2022 at "Cell Death and Differentiation"
DOI: 10.1038/s41418-021-00914-9
Abstract: Huntington’s disease is caused by a pathologically long (>35) CAG repeat located in the first exon of the Huntingtin gene ( HTT ). While pathologically expanded CAG repeats are the focus of extensive investigations, non-pathogenic… read more here.
Keywords: evolutionary history; huntingtin; polyq tract; tract ... See more keywords
Quantitative NMR analysis of the kinetics of prenucleation oligomerization and aggregation of pathogenic huntingtin exon-1 protein
Sign Up to like & getrecommendations! Published in 2022 at "Proceedings of the National Academy of Sciences of the United States of America"
DOI: 10.1073/pnas.2207690119
Abstract: Significance Huntington’s disease is a fatal neurodegenerative condition caused by polyglutamine expansion (≥35) in the N-terminal region of the huntingtin protein encoded by exon-1 (httex1), resulting in fibril accumulation within neuronal inclusion bodies. Microsecond-timescale reversible… read more here.
Keywords: oligomerization; nmr analysis; quantitative nmr; huntingtin ... See more keywords
A comprehensive in silico analysis of huntingtin and its interactome
Sign Up to like & getrecommendations! Published in 2018 at "Journal of Biomolecular Structure and Dynamics"
DOI: 10.1080/07391102.2017.1381646
Abstract: A polyglutamine expansion of the N-terminal region of huntingtin (Htt) causes Huntington’s disease, a severe neurodegenerative disorder. Htt huge multidomain structure, the presence of disordered regions, and the lack of sequence homologs of known structure,… read more here.
Keywords: huntingtin; htt; comprehensive silico; silico analysis ... See more keywords
Small molecule modulator of protein disulfide isomerase attenuates mutant huntingtin toxicity and inhibits endoplasmic reticulum stress in a mouse model of Huntington’s disease
Sign Up to like & getrecommendations! Published in 2018 at "Human Molecular Genetics"
DOI: 10.1093/hmg/ddy061
Abstract: Huntington's disease (HD) is caused by a cytosine-adenine-guanine (CAG) trinucleotide repeat expansion in the huntingtin (HTT) gene encoding an elongated polyglutamine tract within the N-terminal of the huntingtin protein (Htt) and leads to Htt misfolding,… read more here.
Keywords: protein; huntingtin; huntington disease; toxicity ... See more keywords
Characterization of huntingtin interactomes and their dynamic responses in living cells by proximity proteomics
Sign Up to like & getrecommendations! Published in 2022 at "Journal of Neurochemistry"
DOI: 10.1111/jnc.15726
Abstract: Huntingtin (Htt) is a large protein without clearly defined molecular functions. Mutation in this protein causes Huntington's disease (HD), a fatal inherited neurodegenerative disorder. Identification of Htt‐interacting proteins by the traditional approaches including yeast two‐hybrid… read more here.
Keywords: htt; proximity; mutant htt; living cells ... See more keywords