Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …

Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the
neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies …

Herein, we review advances in understanding a group of disorders collectively known as
TAR-DNA binding protein 43 (TDP-43) proteinopathies since the report that TDP-43 is the …

TDP-43 and FUS normal cytoplasmic functions are thought to involve regulated aggregation
and disaggregation [1, 5, 8], similar to that of prion proteins, where aggregation occurs by a …

Ever since the significance of pathological 43-kDa transactivating responsive sequence
DNA-binding protein (TDP-43) for human disease has been recognized in amyotrophic …

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are clinically distinct
fatal neurodegenerative disorders. Increasing molecular evidence indicates that both …

The identification of TDP-43 as the major component of the pathologic inclusions in most
forms of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive …

Dominant mutations in two DNA/RNA binding proteins, TDP-43 and FUS/TLS, are causes of
inherited Amyotrophic Lateral Sclerosis (ALS). TDP-43 and FUS/TLS have striking structural …

The discovery that aggregated transactive response DNA-binding protein 43 kDa (TDP-43)
is the major component of pathological ubiquitinated inclusions in amyotrophic lateral …

Background TDP-43 is a major component of the ubiquitinated inclusions that characterise
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with …