Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …

The pathological process underlying amyotrophic lateral sclerosis (ALS) is associated with
the formation of cytoplasmic inclusions consisting mainly of phosphorylated 43-kDa …

TDP-43 is the major component protein of ubiquitin-positive inclusions in brains of patients
with frontotemporal lobar degeneration (FTLD-TDP) or amyotrophic lateral sclerosis (ALS) …

Frontotemporal dementia (FTD) is a clinically and pathologically heterogeneous group of
non-Alzheimer dementias characterised collectively by relatively selective, progressive …

An expanded GGGGCC repeat in a non-coding region of the C9orf72 gene is a common
cause of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis. Non …

Hexanucleotide repeat expansion in C9ORF72 is the most common genetic cause of
frontotemporal dementia and motor neuron disease. Recently, unconventional non-ATG …

Neurodegenerative diseases are characterised by neuronal loss and cerebral deposition of
proteins with altered physicochemical properties. The major proteins are amyloid-β (Aβ), tau …

Neural networks provide candidate substrates for the spread of proteinopathies causing
neurodegeneration, and emerging data suggest that macroscopic signatures of network …

Genetic analysis revealed the hexanucleotide repeat expansion GGGGCC within the
regulatory region of the gene C9orf72 as the most common cause of familial amyotrophic …

Mutations in SQSTM1 encoding the sequestosome 1/p62 protein have recently been
identified in familial and sporadic cases of amyotrophic lateral sclerosis (ALS). p62 is a …