C9orf72-mediated ALS and FTD: multiple pathways to disease

R Balendra, AM Isaacs - Nature Reviews Neurology, 2018 - nature.com
The discovery that repeat expansions in the C9orf72 gene are a frequent cause of
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) has revolutionized …

Decoding ALS: from genes to mechanism

JP Taylor, RH Brown Jr, DW Cleveland - Nature, 2016 - nature.com
Amyotrophic lateral sclerosis (ALS) is a progressive and uniformly fatal neurodegenerative
disease. A plethora of genetic factors have been identified that drive the degeneration of …

C9ORF72: what it is, what it does, and why it matters

J Smeyers, EG Banchi, M Latouche - Frontiers in cellular …, 2021 - frontiersin.org
When the non-coding repeat expansion in the C9ORF72 gene was discovered to be the
most frequent cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis …

Pathological combinations in neurodegenerative disease are heterogeneous and disease-associated

JL Robinson, SX **e, DR Baer, ER Suh… - Brain, 2023 - academic.oup.com
Pathologies that are causative for neurodegenerative disease (ND) are also frequently
present in unimpaired, older individuals. In this retrospective study of 1647 autopsied …

Motor neuron susceptibility in ALS/FTD

AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …

Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration

EB Lee, S Porta, G Michael Baer, Y Xu, ER Suh… - Acta …, 2017 - Springer
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be
categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to …

Prion-like propagation of protein misfolding and aggregation in amyotrophic lateral sclerosis

L McAlary, SS Plotkin, JJ Yerbury… - Frontiers in molecular …, 2019 - frontiersin.org
The discovery that prion protein can misfold into a pathological conformation that encodes
structural information capable of both propagation and inducing severe neuropathology has …

The expanding biology of the C9orf72 nucleotide repeat expansion in neurodegenerative disease

AR Haeusler, CJ Donnelly, JD Rothstein - Nature Reviews …, 2016 - nature.com
A nucleotide repeat expansion (NRE) within the chromosome 9 open reading frame 72
(C9orf72) gene was the first of this type of mutation to be linked to multiple neurological …