Disruption of axonal transport in neurodegeneration

SH Berth, TE Lloyd - The Journal of Clinical Investigation, 2023 - Am Soc Clin Investig
Neurons are markedly compartmentalized, which makes them reliant on axonal transport to
maintain their health. Axonal transport is important for anterograde delivery of newly …

Huntington's disease: mechanisms of pathogenesis and therapeutic strategies

M Jimenez-Sanchez, F Licitra… - Cold Spring …, 2017 - perspectivesinmedicine.cshlp.org
Huntington's disease is a late-onset neurodegenerative disease caused by a CAG
trinucleotide repeat in the gene encoding the huntingtin protein. Despite its well-defined …

Axonal transport deficits and neurodegenerative diseases

S Millecamps, JP Julien - Nature Reviews Neuroscience, 2013 - nature.com
The intracellular transport of organelles along an axon is crucial for the maintenance and
function of a neuron. Anterograde axonal transport has a role in supplying proteins and …

Altered ribostasis: RNA-protein granules in degenerative disorders

M Ramaswami, JP Taylor, R Parker - Cell, 2013 - cell.com
The molecular processes that contribute to degenerative diseases are not well understood.
Recent observations suggest that some degenerative diseases are promoted by the …

Huntington's disease: from molecular pathogenesis to clinical treatment

CA Ross, SJ Tabrizi - The Lancet Neurology, 2011 - thelancet.com
Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an
expanded CAG repeat in the huntingtin gene, which encodes an abnormally long …

Genetics and neuropathology of Huntington's disease

A Reiner, I Dragatsis, P Dietrich - International review of neurobiology, 2011 - Elsevier
Huntington's disease (HD) is an autosomal dominant progressive neurodegenerative
disorder that prominently affects the basal ganglia, leading to affective, cognitive, behavioral …

The whereabouts of microRNA actions: cytoplasm and beyond

AKL Leung - Trends in cell biology, 2015 - cell.com
MicroRNAs (miRNAs) are a conserved class of approximately 22 nucleotide (nt) short
noncoding RNAs that normally silence gene expression via translational repression and/or …

Huntingtin and the Synapse

JC Barron, EP Hurley, MP Parsons - Frontiers in Cellular …, 2021 - frontiersin.org
Huntington disease (HD) is a monogenic disease that results in a combination of motor,
psychiatric and cognitive symptoms. HD is caused by a CAG trinucleotide repeat expansion …

RNA: The Unsuspected Conductor in the Orchestra of Macromolecular Crowding

E Zacco, L Broglia, M Kurihara, M Monti… - Chemical …, 2024 - ACS Publications
This comprehensive Review delves into the chemical principles governing RNA-mediated
crowding events, commonly referred to as granules or biological condensates. We explore …

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 …