Forms and Phases in Huntingtin Protein Aggregation. [PDF]
Molecular Cell, 2018 Using a combination of fluorescence microscopy and electron tomography, Peskett et al. (2018), in this issue of Molecular Cell, explore the nucleation of amyloid-like filaments from liquid-like condensates of huntingtin protein exon1 with disease-related polyQ extensions.
M. Elbaum
semanticscholar +3 more sources
Phosphorylation of huntingtin at residue T3 is decreased in Huntington's disease and modulates mutant huntingtin protein conformation. [PDF]
Proc Natl Acad Sci U S A, 2017 Significance The findings in this manuscript report on the identification of a posttranslational modification in the huntingtin protein (phosphorylation on residue T3 in the N17 region of the protein), which can revert the conformational effects of the ...
Cariulo C +16 more
europepmc +9 more sources
The predicted structure of the headpiece of the Huntingtin protein and its implications on Huntingtin aggregation. [PDF]
Journal of Molecular Biology, 2009 We have performed simulated tempering molecular dynamics simulations to study the thermodynamics of the headpiece of the Huntingtin (Htt) protein (N17(Htt)). With converged sampling, we found this peptide is highly helical, as previously proposed. Interestingly, this peptide is also found to adopt two different and seemingly stable states.
N. W. Kelley +5 more
semanticscholar +5 more sources
Frontiers in Cellular Neuroscience, 2020 Huntingtin-associated protein 1 (Hap1) was initially identified as a brain-enriched protein that binds to the Huntington’s disease protein, huntingtin. Unlike huntingtin that is ubiquitously expressed in the brain, Hap1 is enriched in the brain with the ...
Xingxing Chen +13 more
doaj +2 more sources
Inhibition of mitochondrial protein import by mutant huntingtin [PDF]
Nature Neuroscience, 2014 Mitochondrial dysfunction is associated with neuronal loss in Huntington's disease (HD), a neurodegenerative disease caused by an abnormal polyglutamine expansion in huntingtin (Htt). However, the mechanisms linking mutant Htt and mitochondrial dysfunction in HD remain unknown.
H. Yano +9 more
semanticscholar +4 more sources
Potential function for the Huntingtin protein as a scaffold for selective autophagy. [PDF]
Proc Natl Acad Sci U S A, 2014 Significance The normal function of the Huntingtin (HTT) protein is emerging. Here we report that selective autophagy requires an intact HTT protein in Drosophila and mouse CNS. We describe similarities in structure and binding activity between the C-terminal domain of HTT and the yeast autophagy scaffold ...
Ochaba J +17 more
europepmc +8 more sources
Lowering mutant huntingtin protein [PDF]
Nature Reviews Drug Discovery, 2019 Sarah Crunkhorn
semanticscholar +3 more sources
The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins [PDF]
Cells, 2021 Protein misfolding and aggregation are implicated in many neurodegenerative diseases. One of these diseases is Huntington’s, which is caused by increased glutamine-encoding trinucleotide repeats within the Huntingtin gene.
Austin Folger, Yanchang Wang
doaj +3 more sources
Detection of antibodies against the huntingtin protein in human plasma. [PDF]
Cell Mol Life Sci, 2023 AbstractHuntington’s disease (HD) is a dominantly inherited neurodegenerative disorder resulting from a CAG expansion in the huntingtin (HTT) gene, which leads to the production and accumulation of mutant huntingtin (mHTT). While primarily considered a disorder of the central nervous system, multiple changes have been described to occur throughout the ...
Denis HL +10 more
europepmc +5 more sources
p62/SQSTM1 forms protein aggregates degraded by autophagy and has a protective effect on huntingtin-induced cell death [PDF]
Journal of Cell Biology, 2005 Autophagic degradation of ubiquitinated protein aggregates is important for cell survival, but it is not known how the autophagic machinery recognizes such aggregates.
Geir Bjørkøy +7 more
openalex +2 more sources