Results 31 to 40 of about 5,553,804 (280)
Mutant Huntingtin Protein Measurement
Definitions, 2020 The determination of the amount of mutant huntingtin protein present in a sample.
semanticscholar +1 more source
Nature Communications, 2021 The polyglutamine expansion of huntingtin (mHTT) causes Huntington disease (HD) and neurodegeneration, but the mechanisms remain unclear. Here, we found that mHtt promotes ribosome stalling and suppresses protein synthesis in mouse HD striatal neuronal ...
M. Eshraghi +12 more
semanticscholar +1 more source
Stimulation of NeuroD activity by huntingtin and huntingtin-associated proteins HAP1 and MLK2 [PDF]
Proceedings of the National Academy of Sciences, 2003 NeuroD (ND) is a basic helix–loop–helix transcription factor important for neuronal development and survival. By using a yeast two-hybrid screen, we identified two proteins that interact with ND, huntingtin-associated protein 1 (HAP1) and mixed-lineage kinase 2 (MLK2), both of which are known to interact with huntingtin (Htt).
Katherine Gowan +2 more
openaire +2 more sources
Huntingtin: A Protein with a Peculiar Solvent Accessible Surface
International Journal of Molecular Sciences, 2021 Taking advantage of the last cryogenic electron microscopy structure of human huntingtin, we explored with computational methods its physicochemical properties, focusing on the solvent accessible surface of the protein and highlighting a quite interesting mix of hydrophobic and hydrophilic patterns, with the prevalence of the latter ones.
Pier Luigi Martelli +4 more
openaire +3 more sources
The pathobiology of perturbed mutant huntingtin protein–protein interactions in Huntington's disease
Journal of Neurochemistry, 2019 Mutations are at the root of many human diseases. Still, we largely do not exactly understand how they trigger pathogenesis. One, more recent, hypothesis has been that they comprehensively perturb protein–protein interaction (PPI) networks and ...
E. Wanker +4 more
semanticscholar +1 more source
Molecular mechanisms of heterogeneous oligomerization of huntingtin proteins [PDF]
Scientific Reports, 2019 AbstractThere is still no successful strategy to treat Huntington’s disease, an inherited autosomal disorder associated with the aggregation of mutated forms of the huntingtin protein containing polyglutamine tracts with more than 36 repeats. Recent experimental evidence is challenging the conventional view of the disease by revealing transcellular ...
S. Bonfanti +7 more
openaire +4 more sources
Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity [PDF]
, 2008 Although expanded polyglutamine (polyQ) repeats are inherently toxic, causing at least nine neurodegenerative diseases, the protein context determines which neurons are affected.
Bugg, Charles W. +5 more
core +2 more sources
Imbalance of p75(NTR)/TrkB protein expression in Huntington's disease: Implication for neuroprotective therapies [PDF]
, 2013 Neuroprotective therapies based on brain-derived neurotrophic factor (BDNF) administration have been proposed for Huntington's disease (HD) treatment. However, our group has recently reported reduced levels of TrkB in HD mouse models and HD human brain ...
Alberch, J. +5 more
core +1 more source
PLoS ONE, 2014 Huntington disease is an adult onset neurodegenerative disease characterized by motor, cognitive, and psychiatric dysfunction, caused by a CAG expansion in the HTT gene. Huntingtin Interacting Protein 14 (HIP14) and Huntingtin Interacting Protein 14-like (HIP14L) are palmitoyl acyltransferases (PATs), enzymes that mediate the post-translational ...
Shaun S. Sanders +3 more
openaire +5 more sources