Huntington disease: new insights into molecular pathogenesis and therapeutic opportunities

SJ Tabrizi, MD Flower, CA Ross, EJ Wild - Nature Reviews Neurology, 2020 - nature.com
Huntington disease (HD) is a neurodegenerative disease caused by CAG repeat expansion
in the huntingtin gene (HTT) and involves a complex web of pathogenic mechanisms …

The cytoplasmic dynein transport machinery and its many cargoes

SL Reck-Peterson, WB Redwine, RD Vale… - … reviews Molecular cell …, 2018 - nature.com
Cytoplasmic dynein 1 is an important microtubule-based motor in many eukaryotic cells.
Dynein has critical roles both in interphase and during cell division. Here, we focus on …

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 …

The biology of huntingtin

F Saudou, S Humbert - Neuron, 2016 - cell.com
Huntingtin (HTT) is now a famous protein because an abnormal expansion of a glutamine
stretch (polyQ) in its N-terminal sequence leads to the devastating neurodegenerative …

Cellular specificity of NF-κB function in the nervous system

EC Dresselhaus, MK Meffert - Frontiers in immunology, 2019 - frontiersin.org
Nuclear Factor Kappa B (NF-κB) is a ubiquitously expressed transcription factor with key
functions in a wide array of biological systems. While the role of NF-κB in processes, such as …

Autophagy induction as a therapeutic strategy for neurodegenerative diseases

A Djajadikerta, S Keshri, M Pavel, R Prestil… - Journal of molecular …, 2020 - Elsevier
Autophagy is a major, conserved cellular pathway by which cells deliver cytoplasmic
contents to lysosomes for degradation. Genetic studies have revealed extensive links …

Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1

DJ Klionsky, AK Abdel-Aziz, S Abdelfatah, M Abdellatif… - autophagy, 2021 - Taylor & Francis
In 2008, we published the first set of guidelines for standardizing research in autophagy.
Since then, this topic has received increasing attention, and many scientists have entered …

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 …

Mitochondrial transport in neurons: impact on synaptic homeostasis and neurodegeneration

ZH Sheng, Q Cai - Nature Reviews Neuroscience, 2012 - nature.com
Mitochondria have a number of essential roles in neuronal function. Their complex mobility
patterns within neurons are characterized by frequent changes in direction. Mobile …