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 …

Protein folding alterations in amyotrophic lateral sclerosis

S Parakh, JD Atkin - Brain research, 2016 - Elsevier
Protein misfolding leads to the formation of aggregated proteins and protein inclusions,
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 …

[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 …

Proteasome activation: an innovative promising approach for delaying aging and retarding age-related diseases

N Chondrogianni, K Voutetakis, M Kapetanou… - Ageing research …, 2015 - Elsevier
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 …

Sequence composition of disordered regions fine-tunes protein half-life

S Fishbain, T Inobe, E Israeli, S Chavali, H Yu… - Nature structural & …, 2015 - nature.com
The proteasome controls the concentrations of most proteins in eukaryotic cells. It
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 …

Monomeric huntingtin exon 1 has similar overall structural features for wild-type and pathological polyglutamine lengths

JB Warner IV, KM Ruff, PS Tan, EA Lemke… - Journal of the …, 2017 - ACS Publications
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 …

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 …

Cell rearrangement and oxidant/antioxidant imbalance in huntington's disease

F D'Egidio, V Castelli, A Cimini, M d'Angelo - Antioxidants, 2023 - mdpi.com
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 …