The many faces of autophagy dysfunction in Huntington's disease: from mechanism to therapy
CJ Cortes, AR La Spada - Drug discovery today, 2014 - Elsevier
Autophagy is the cellular process by which proteins, macromolecules, and organelles are
targeted to and degraded by the lysosome. Given that neurodegenerative diseases involve …
targeted to and degraded by the lysosome. Given that neurodegenerative diseases involve …
Protein folding alterations in amyotrophic lateral sclerosis
Protein misfolding leads to the formation of aggregated proteins and protein inclusions,
which are associated with synaptic loss and neuronal death in neurodegenerative diseases …
which are associated with synaptic loss and neuronal death in neurodegenerative diseases …
Developing therapies for neurodegenerative disorders: insights from protein aggregation and cellular stress responses
GR Mallucci, D Klenerman… - Annual Review of Cell …, 2020 - annualreviews.org
As the world's population ages, neurodegenerative disorders are poised to become the
commonest cause of death. Despite this, they remain essentially untreatable. Characterized …
commonest cause of death. Despite this, they remain essentially untreatable. Characterized …
[PDF][PDF] Huntingtin inclusions trigger cellular quiescence, deactivate apoptosis, and lead to delayed necrosis
YM Ramdzan, MM Trubetskov, AR Ormsby… - Cell reports, 2017 - cell.com
Competing models exist in the literature for the relationship between mutant Huntingtin exon
1 (Httex1) inclusion formation and toxicity. In one, inclusions are adaptive by sequestering …
1 (Httex1) inclusion formation and toxicity. In one, inclusions are adaptive by sequestering …
Proteasome activation: an innovative promising approach for delaying aging and retarding age-related diseases
Aging is a natural process accompanied by a progressive accumulation of damage in all
constituent macromolecules (nucleic acids, lipids and proteins). Accumulation of damage in …
constituent macromolecules (nucleic acids, lipids and proteins). Accumulation of damage in …
Sequence composition of disordered regions fine-tunes protein half-life
The proteasome controls the concentrations of most proteins in eukaryotic cells. It
recognizes its protein substrates through ubiquitin tags and initiates degradation at …
recognizes its protein substrates through ubiquitin tags and initiates degradation at …
[HTML][HTML] Targeting the proteostasis network in Huntington's disease
TR Soares, SD Reis, BR Pinho, MR Duchen… - Ageing Research …, 2019 - Elsevier
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by
a polyglutamine expansion mutation in the huntingtin protein. Expansions above 40 …
a polyglutamine expansion mutation in the huntingtin protein. Expansions above 40 …
Monomeric huntingtin exon 1 has similar overall structural features for wild-type and pathological polyglutamine lengths
Huntington's disease is caused by expansion of a polyglutamine (polyQ) domain within exon
1 of the huntingtin gene (Httex1). The prevailing hypothesis is that the monomeric Httex1 …
1 of the huntingtin gene (Httex1). The prevailing hypothesis is that the monomeric Httex1 …
Neurodegenerative diseases linked to misfolded proteins and their therapeutic approaches: A review
H Khanam, A Ali, M Asif - European journal of medicinal chemistry, 2016 - Elsevier
Neurodegenerative diseases, such as Alzheimer's, Parkinson's, Creutzfeldt–Jacob,
Huntington's diseases and amyotrophic lateral sclerosis, are mainly characterized by the …
Huntington's diseases and amyotrophic lateral sclerosis, are mainly characterized by the …
Cell rearrangement and oxidant/antioxidant imbalance in huntington's disease
Huntington's Disease (HD) is a hereditary neurodegenerative disorder caused by the
expansion of a CAG triplet repeat in the HTT gene, resulting in the production of an aberrant …
expansion of a CAG triplet repeat in the HTT gene, resulting in the production of an aberrant …