ALS: Stress Granules

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by cytoplasmic protein aggregates within motor neurons. TDP-43 is an ALS-linked protein that is known to regulate splicing and storage of specific mRNAs into stress granules, which have been implicated in formation of ALS protein aggregates. Here is the latest research in this field.

February 20, 2021
Preprint
Open Access

Nup62 is recruited to pathological condensates and promotes TDP-43 insolubility in C9orf72 and sporadic ALS/FTLD.

ResearchSquare
Amanda GleixnerChristopher Donnelly
February 13, 2021
Open Access

Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression.

Neurobiology of Disease
Júlia Canet-PonsGeorg Auburger
February 5, 2021
Open Access

NMR Characterization of Angiogenin Variants and tRNAAla Products Impacting Aberrant Protein Oligomerization.

International Journal of Molecular Sciences
Andrea FagagniniDouglas Laurents
February 3, 2021
Preprint
Open Access

DEFINING THE DIVERSITY OF HNRNPA1 MUTATIONS IN CLINICAL PHENOTYPE AND PATHOMECHANISM

MedRxiv : the Preprint Server for Health Sciences
D. BeijerJonathan Baets
January 31, 2021
Review
Open Access

From Prions to Stress Granules: Defining the Compositional Features of Prion-Like Domains That Promote Different Types of Assemblies.

International Journal of Molecular Sciences
Anastasia Fomicheva, Eric D Ross
December 24, 2020

FUS regulates autophagy by mediating the transcription of genes critical to the autophagosome formation.

Journal of Neurochemistry
Alexandra ArenasHaining Zhu
January 18, 2021
Open Access

AggreCount: an unbiased image analysis tool for identifying and quantifying cellular aggregates in a spatially defined manner.

The Journal of Biological Chemistry
J. A. KlicksteinMalavika Raman
January 16, 2021
Review

FUS and TDP-43 Phases in Health and Disease.

Trends in Biochemical Sciences
Bede PortzJames Shorter
January 9, 2021

NMR assignments for the C-terminal domain of human TDP-43

Biomolecular NMR Assignments
David Pantoja-UcedaMiguel Mompeán
January 8, 2021
Preprint
Open Access

Identification of hub molecules of FUS-ALS by Bayesian gene regulatory network analysis of iPSC model: iBRN

BioRxiv : the Preprint Server for Biology
M. NogamiHideyuki Okano
January 8, 2021
Preprint
Open Access

Enhanced detection of nucleotide repeat mRNA with hybridization chain reaction

BioRxiv : the Preprint Server for Biology
M. R. GlineburgPeter Todd
January 8, 2021

Cytoplasmic granule formation by FUS-R495X is attributable to arginine methylation in all Gly-rich, RGG1 and RGG2 domains.

Genes to Cells : Devoted to Molecular & Cellular Mechanisms
Daiki KawaharaTadashi Nakaya
January 5, 2021
Preprint
Open Access

NMR Characterization of Angiogenin Variants and tRNAAla Products Impacting Aberrant Protein Oligomerization

Preprints.org
Andrea FagagniniDouglas Laurents
December 29, 2020
Open Access

LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient

PLoS Biology
Jongbo LeeChunghun Lim
December 8, 2020
Review
Open Access

Stress Granule Dysregulation in Amyotrophic Lateral Sclerosis

Frontiers in Cellular Neuroscience
Jessica Dudman, Xin Qi
November 5, 2020
Review

Multisystem proteinopathy: Where myopathy and motor neuron disease converge

Muscle & Nerve
Manisha K KorbTahseen Mozaffar
November 1, 2020
Review
Open Access

Maintaining the balance of TDP-43, mitochondria, and autophagy: a promising therapeutic strategy for neurodegenerative diseases

Translational Neurodegeneration
Chunhui HuangZaijun Zhang
October 23, 2020
Open Access

ANXA11 mutations in ALS cause dysregulation of calcium homeostasis and stress granule dynamics.

Science Translational Medicine
Minyeop NahmSeung Hyun Kim
October 22, 2020
Open Access

AggreCount: An unbiased image analysis tool for identifying and quantifying cellular aggregates in a spatially-defined manner.

The Journal of Biological Chemistry
J. A. KlicksteinMalavika Raman

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