Analysis of Huntington's disease modifiers using the hyperbolic mapping of the protein interaction network

AC Vagiona, P Mier, S Petrakis… - International Journal of …, 2022 - mdpi.com
Huntington's disease (HD) is caused by the production of a mutant huntingtin (HTT) with an
abnormally long poly-glutamine (polyQ) tract, forming aggregates and inclusions in neurons …

Aggregation behavior of chemically synthesized, full-length huntingtin exon1

B Sahoo, D Singer, R Kodali, T Zuchner, R Wetzel - Biochemistry, 2014 - ACS Publications
Repeat length disease thresholds vary among the 10 expanded polyglutamine (polyQ)
repeat diseases, from about 20 to about 50 glutamine residues. The unique amino acid …

The emerging role of the first 17 amino acids of huntingtin in Huntington's disease

JR Arndt, M Chaibva, J Legleiter - Biomolecular concepts, 2015 - degruyter.com
Huntington's disease (HD) is caused by a polyglutamine (polyQ) domain that is expanded
beyond a critical threshold near the N-terminus of the huntingtin (htt) protein, directly leading …

A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure

MA Poirier, H Jiang, CA Ross - Human molecular genetics, 2005 - academic.oup.com
Huntington's disease (HD) arises from an expanded polyglutamine (polyQ) in the N-terminus
of the huntingtin (htt) protein. Neuronal degeneration and inclusions containing N-terminal …

Biophysical aspect of huntingtin protein during polyQ: An in silico insight

C Gopalakrishnan, S Jethi, N Kalsi… - Cell Biochemistry and …, 2016 - Springer
Huntington's disease (HD) is a neurodegenerative disorder that is caused by an abnormal
elongation of the polyglutamine (polyQ) chain in the Huntington (Htt) protein. At present, the …

Putting huntingtin “aggregation” in view with windows into the cellular milieu

DM Hatters - Current topics in medicinal chemistry, 2012 - ingentaconnect.com
Huntington's disease arises from CAG codon-repeat expansions in the Htt gene, which
leads to a Htt gene product with an expanded polyglutamine (polyQ) sequence. The length …

Aggregation landscapes of Huntingtin exon 1 protein fragments and the critical repeat length for the onset of Huntington's disease

M Chen, PG Wolynes - … of the National Academy of Sciences, 2017 - National Acad Sciences
Huntington's disease (HD) is a neurodegenerative disease caused by an abnormal
expansion in the polyglutamine (polyQ) track of the Huntingtin (HTT) protein. The severity of …

Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions

A Davranche, H Aviolat, G Zeder-Lutz… - Human molecular …, 2011 - academic.oup.com
Huntington's disease (HD) is caused by the expansion mutation above a length threshold of
a polyglutamine (polyQ) stretch in the huntingtin (Htt) protein. Mutant Htt (mHtt) pathogenicity …

A method for the analysis of the oligomerization profile of the Huntington's disease-associated, aggregation-prone mutant huntingtin protein by isopycnic …

R Bonavita, R Di Martino, G Cortone… - Frontiers in Molecular …, 2024 - frontiersin.org
Conformational diseases, such as Alzheimer's, Parkinson's and Huntington's diseases as
well as ataxias and fronto-temporal disorders, are part of common class of neurological …

Multiple discrete soluble aggregates influence polyglutamine toxicity in a Huntington's disease model system

W Xi, X Wang, TM Laue, CL Denis - Scientific reports, 2016 - nature.com
Huntington's disease (HD) results from expansions of polyglutamine stretches (polyQ) in the
huntingtin protein (Htt) that promote protein aggregation, neurodegeneration, and death …