Results 21 to 30 of about 37,064 (267)
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).
Edoardo, Marcora +2 more
openaire +2 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.
Kelley, Nicholas W. +5 more
openaire +3 more sources
Frontiers in Physiology, 2023 Introduction: Huntington disease is an autosomal dominant neurodegenerative disorder which is caused by a CAG repeat expansion in the HTT gene that codes for an elongated polyglutamine tract in the huntingtin (HTT) protein.
Fanny L. Lemarié +4 more
doaj +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 ...
Silvia Bonfanti +7 more
openalex +5 more sources
Cells, 2020 Huntington’s disease is a severe and currently incurable neurodegenerative disease. An autosomal dominant mutation in the Huntingtin gene (HTT) causes an increase in the polyglutamine fragment length at the protein N-terminus.
Aleksandra S. Taran +3 more
doaj +1 more source
Detection of ubiquitinated huntingtin species in intracellular aggregates
Frontiers in Molecular Neuroscience, 2015 Protein conformation diseases, including polyglutamine diseases, result from the accumulation and aggregation of misfolded proteins. Huntington’s disease is one of nine diseases caused by an expanded polyglutamine repeat within the affected protein and ...
Katrin eJuenemann +2 more
doaj +1 more source
Evidence for dynamic and multiple roles for huntingtin in Ciona intestinalis [PDF]
, 2013 Although mutations in the huntingtin gene (HTT) due to poly-Q expansion cause neuropathology in humans (Huntington's disease; HD), the normal function(s) of the gene and its protein (HTT) remain obscure.
Brown, Euan R. +2 more
core +1 more source
Biomedicines, 2023 Brain-derived neurotrophic factor (BDNF) is a major neurotrophin whose loss or interruption is well established to have numerous intersections with the pathogenesis of progressive neurological disorders.
Andrew Speidell +2 more
doaj +1 more source
Disease Models & Mechanisms, 2014 Huntingtin is a large membrane-associated scaffolding protein that associates with endocytic and exocytic vesicles and modulates their trafficking along cytoskeletal tracks. Although the progression of Huntington’s disease is linked to toxic accumulation
Hemma Brandstaetter +2 more
doaj +1 more source
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