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The flavonoid luteolin reduces mutant huntingtin aggregation and cytotoxicity in huntingtin-mutated neuroblastoma cells
Published 2023-12-01“…Background: Huntington's disease is an inherited progressive neurodegenerative disorder caused by an expansion of the polyglutamine tract leading to malformation and aggregation of the mutant huntingtin protein in the cell cytoplasm and nucleus of affected brain regions. …”
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Huntingtin Ubiquitination Mechanisms and Novel Possible Therapies to Decrease the Toxic Effects of Mutated Huntingtin
Published 2021-12-01Subjects: Get full text
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Huntingtin exon 1 deletion does not alter the subcellular distribution of huntingtin and gene transcription in mice
Published 2022-11-01“…Huntington disease (HD) is caused by the expansion of CAG triplet repeats in exon 1 of the huntingtin (HTT) gene, which also encodes the first 17 amino acids (N-17) that can modulate the toxicity of the expanded polyQ repeat. …”
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A pathogenic proteolysis–resistant huntingtin isoform induced by an antisense oligonucleotide maintains huntingtin function
Published 2022-09-01“…Its key pathological mechanism involves the proteolysis of polyglutamine-expanded (polyQ-expanded) mutant huntingtin (mHTT), which generates N-terminal fragments containing polyQ, a key contributor to HD pathogenesis. …”
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Quantifying mutant huntingtin protein in human cerebrospinal fluid to support the development of huntingtin-lowering therapies
Published 2023-04-01“…Abstract Huntington’s disease (HD) is caused by a cytosine adenine guanine-repeat expansion in the huntingtin gene. This results in the production of toxic mutant huntingtin protein (mHTT), which has an elongated polyglutamine (polyQ) stretch near the protein’s N-terminal end. …”
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Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1
Published 2022-09-01Subjects: “…huntingtin…”
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Huntingtin Interacting Proteins and Pathological Implications
Published 2023-08-01Subjects: “…huntingtin…”
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Detection of ubiquitinated huntingtin species in intracellular aggregates
Published 2015-01-01Subjects: Get full text
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In Silico Analysis of Huntingtin Homologs in Lower Eukaryotes
Published 2021-03-01Subjects: “…huntingtin…”
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Unconventional Secretion and Intercellular Transfer of Mutant Huntingtin
Published 2018-06-01Subjects: “…Huntingtin (HTT)…”
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The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins
Published 2021-10-01Subjects: Get full text
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Correction: Functions of Huntingtin in Germ Layer Specification and Organogenesis
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Random Lasing Detection of Mutant Huntingtin Expression in Cells
Published 2021-05-01“…Huntington’s disease (HD) is an autosomal dominant, incurable neurodegenerative disease caused by mutation in the huntingtin gene (<i>HTT</i>). <i>HTT</i> mutation leads to protein misfolding and aggregation, which affect cells’ functions and structural features. …”
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Correction: Functions of Huntingtin in Germ Layer Specification and Organogenesis.
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Huntingtin and Its Role in Mechanisms of RNA-Mediated Toxicity
Published 2021-07-01Subjects: “…huntingtin…”
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Characterization of axonal transport defects in Drosophila Huntingtin mutants
Published 2016“…Polyglutamine (polyQ) expansion within Huntingtin (Htt) causes the fatal neurodegenerative disorder Huntington’s Disease (HD). …”
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Behavioral features in child and adolescent huntingtin gene‐mutation carriers
Published 2022-07-01“…Abstract Introduction We compared neuropsychiatric symptoms between child and adolescent huntingtin gene‐mutation carriers and noncarriers. Given previous evidence of atypical striatal development in carriers, we also assessed the relationship between neuropsychiatric traits and striatal development. …”
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Cyclodextrin-Based Nanoparticles for Delivery of Antisense Oligonucleotides Targeting Huntingtin
Published 2023-02-01“…Huntington’s disease (HD) is a progressive inherited neurodegenerative disease caused by a CAG repeat expansion in the huntingtin gene, which is translated into the pathologic mutant huntingtin (mHTT) protein. …”
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