Limbic-predominant age-related TDP-43 encephalopathy (LATE): consensus working group report

PT Nelson, DW Dickson, JQ Trojanowski, CR Jack… - Brain, 2019 - academic.oup.com
We describe a recently recognized disease entity, limbic-predominant age-related TDP-43
encephalopathy (LATE). LATE neuropathological change (LATE-NC) is defined by a …

ALS genetics, mechanisms, and therapeutics: where are we now?

R Mejzini, LL Flynn, IL Pitout, S Fletcher… - Frontiers in …, 2019 - frontiersin.org
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …

Pathology of neurodegenerative diseases

BN Dugger, DW Dickson - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Neurodegenerative disorders are characterized by progressive loss of selectively vulnerable
populations of neurons, which contrasts with select static neuronal loss because of …

Structure of pathological TDP-43 filaments from ALS with FTLD

D Arseni, M Hasegawa, AG Murzin, F Kametani, M Arai… - Nature, 2022 - nature.com
The abnormal aggregation of TAR DNA-binding protein 43 kDa (TDP-43) in neurons and
glia is the defining pathological hallmark of the neurodegenerative disease amyotrophic …

Molecular pathology of neurodegenerative diseases by cryo-EM of amyloids

SHW Scheres, B Ryskeldi-Falcon, M Goedert - Nature, 2023 - nature.com
Abnormal assembly of tau, α-synuclein, TDP-43 and amyloid-β proteins into amyloid
filaments defines most human neurodegenerative diseases. Genetics provides a direct link …

Self-propagation of pathogenic protein aggregates in neurodegenerative diseases

M Jucker, LC Walker - Nature, 2013 - nature.com
For several decades scientists have speculated that the key to understanding age-related
neurodegenerative disorders may be found in the unusual biology of the prion diseases …

Amyotrophic lateral sclerosis-frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria

MJ Strong, S Abrahams, LH Goldstein… - … lateral sclerosis and …, 2017 - Taylor & Francis
This article presents the revised consensus criteria for the diagnosis of frontotemporal
dysfunction in amyotrophic lateral sclerosis (ALS) based on an international research …

Alzheimer's and Parkinson's diseases: The prion concept in relation to assembled Aβ, tau, and α-synuclein

M Goedert - Science, 2015 - science.org
BACKGROUND Alzheimer's disease (AD) and Parkinson's disease (PD) are the most
common human neurodegenerative diseases. AD is primarily a dementing disease, and PD …

Selective vulnerability in neurodegenerative diseases

H Fu, J Hardy, KE Duff - Nature neuroscience, 2018 - nature.com
Neurodegenerative diseases have two general characteristics that are so fundamental we
usually take them for granted. The first is that the pathology associated with the disease only …

TDP-43 proteinopathies: a new wave of neurodegenerative diseases

EMJ De Boer, VK Orie, T Williams, MR Baker… - Journal of Neurology …, 2021 - jnnp.bmj.com
Inclusions of pathogenic deposits containing TAR DNA-binding protein 43 (TDP-43) are
evident in the brain and spinal cord of patients that present across a spectrum of …