Results 71 to 80 of about 19,301 (228)
Annals of Neurology, EarlyView.Objective An enduring puzzle in many inherited neurological disorders is the late onset of symptoms despite expression of function‐impairing mutant protein early in life. We examined the basis for onset of impairment in spinocerebellar ataxia type 6 (SCA6), a canonical late‐onset neurodegenerative ataxia which results from a polyglutamine expansion in ...
Haoran Huang +10 more
wiley +1 more source
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
Novel DNA Aptamers that Bind to Mutant Huntingtin and Modify Its Activity
Molecular Therapy: Nucleic Acids, 2018 The CAG repeat expansion that elongates the polyglutamine tract in huntingtin is the root genetic cause of Huntington’s disease (HD), a debilitating neurodegenerative disorder.
Baehyun Shin +12 more
doaj +1 more source
Nanomaterial‐based immune therapeutic strategies in neurodegenerative diseases
BMEMat, EarlyView.This review highlights the immunomodulatory potential of nanomaterials (NMs) in treating neurodegenerative diseases (NDs). It focuses on their roles in regulating innate and adaptive immune responses to maintain immune homeostasis. By providing insights into these mechanisms, the review lays the groundwork for innovative NMs therapeutic strategies to ...
Xinru Zhou +6 more
wiley +1 more source
Molecular Neurodegeneration, 2010 Background Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a CAG repeat expansion within the huntingtin gene. Mutant huntingtin protein misfolds and accumulates within neurons where it mediates its toxic effects. Promoting
Frentzel Stefan +11 more
doaj +1 more source
The Cytotoxicity and Clearance of Mutant Huntingtin and Other Misfolded Proteins
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 +1 more source
The power of many: when genetics met yeasts and high‐throughput
Biological Reviews, EarlyView.ABSTRACT In recent years, complex technological capabilities have evolved, driven by the need to solve complex and integrative biological questions through global analyses. New equipment allows the scaling up and automation of processes which previously were carried out on a very limited scale.
Víctor A. Tallada, Víctor Carranco
wiley +1 more source
Mechanisms of copper ion mediated Huntington's disease progression. [PDF]
PLoS ONE, 2007 Huntington's disease (HD) is caused by a dominant polyglutamine expansion within the N-terminus of huntingtin protein and results in oxidative stress, energetic insufficiency and striatal degeneration.
Jonathan H Fox +9 more
doaj +1 more source
Huntingtin-associated protein 1 ameliorates neurological function rehabilitation by facilitating neurite elongation through TrKA-MAPK pathway in mice spinal cord injury [PDF]
, 2023 Miao Li, Sun Wan Qing, Tao Lu
openalex +1 more source
Mitochondria and the Actin Cytoskeleton in Neurodegeneration
Cytoskeleton, EarlyView.ABSTRACT Mitochondrial dysfunction and cytoskeletal disorganization are widely recognized hallmarks of neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS).
Shivani Tuli +3 more
wiley +1 more source