Stress granules and neurodegeneration

B Wolozin, P Ivanov - Nature Reviews Neuroscience, 2019 - nature.com
Recent advances suggest that the response of RNA metabolism to stress has an important
role in the pathophysiology of neurodegenerative diseases, particularly amyotrophic lateral …

Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

[HTML][HTML] RNA granules hitchhike on lysosomes for long-distance transport, using annexin A11 as a molecular tether

YC Liao, MS Fernandopulle, G Wang, H Choi, L Hao… - Cell, 2019 - cell.com
Long-distance RNA transport enables local protein synthesis at metabolically-active sites
distant from the nucleus. This process ensures an appropriate spatial organization of …

Axonal transport and neurological disease

JN Sleigh, AM Rossor, AD Fellows… - Nature Reviews …, 2019 - nature.com
Axonal transport is the process whereby motor proteins actively navigate microtubules to
deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is …

ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair

JR Klim, LA Williams, F Limone… - Nature …, 2019 - nature.com
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its …

Autophagy in neurons

AKH Stavoe, ELF Holzbaur - Annual review of cell and …, 2019 - annualreviews.org
Autophagy is the major cellular pathway to degrade dysfunctional organelles and protein
aggregates. Autophagy is particularly important in neurons, which are terminally …

Axonal transport: Driving synaptic function

P Guedes-Dias, ELF Holzbaur - Science, 2019 - science.org
BACKGROUND Neurons are polarized cells with extreme geometries. Multiple dendrites
and one axon generally emerge from a single cell body and establish synaptic contacts with …

Postmortem cortex samples identify distinct molecular subtypes of ALS: retrotransposon activation, oxidative stress, and activated glia

OH Tam, NV Rozhkov, R Shaw, D Kim, I Hubbard… - Cell reports, 2019 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the progressive loss of motor neurons. While several pathogenic mutations have been …

Axonal transport and mitochondrial function in neurons

A Mandal, CM Drerup - Frontiers in Cellular Neuroscience, 2019 - frontiersin.org
The complex and elaborate architecture of a neuron poses a great challenge to the cellular
machinery which localizes proteins and organelles, such as mitochondria, to necessary …

Shared polygenic risk and causal inferences in amyotrophic lateral sclerosis

S Bandres‐Ciga, AJ Noyce, G Hemani… - Annals of …, 2019 - Wiley Online Library
Objective To identify shared polygenic risk and causal associations in amyotrophic lateral
sclerosis (ALS). Methods Linkage disequilibrium score regression and Mendelian …