Frontotemporal dementia
NT Olney, S Spina, BL Miller - Neurologic clinics, 2017 - neurologic.theclinics.com
Frontotemporal dementia (FTD) has undergone numerous changes in nomenclature and
categorization schemes since it was first described by Pick in 1892. Presently, FTD …
categorization schemes since it was first described by Pick in 1892. Presently, FTD …
RNA-binding proteins with prion-like domains in health and disease
AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
FUS phase separation is modulated by a molecular chaperone and methylation of arginine cation-π interactions
Reversible phase separation underpins the role of FUS in ribonucleoprotein granules and
other membrane-free organelles and is, in part, driven by the intrinsically disordered low …
other membrane-free organelles and is, in part, driven by the intrinsically disordered low …
Clinicopathological correlations in behavioural variant frontotemporal dementia
Accurately predicting the underlying neuropathological diagnosis in patients with
behavioural variant frontotemporal dementia (bvFTD) poses a daunting challenge for …
behavioural variant frontotemporal dementia (bvFTD) poses a daunting challenge for …
Molecular neuropathology of frontotemporal dementia: insights into disease mechanisms from postmortem studies
Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular
basis. The past decade has seen the discovery of several new FTD‐causing genetic …
basis. The past decade has seen the discovery of several new FTD‐causing genetic …
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia
Amyotrophic lateral sclerosis (ALS) is a paralytic and usually fatal disorder caused by motor-
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …
neuron degeneration in the brain and spinal cord. Most cases of ALS are sporadic but about …
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease
Prions are self-templating protein conformers that are naturally transmitted between
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
Genetic mutations in RNA-binding proteins and their roles in ALS
Mutations in genes that encode RNA-binding proteins (RBPs) have emerged as critical
determinants of neurological diseases, especially motor neuron disorders such as …
determinants of neurological diseases, especially motor neuron disorders such as …
Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment
LH Goldstein, S Abrahams - The Lancet Neurology, 2013 - thelancet.com
Increased awareness of cognitive and behavioural change in amyotrophic lateral sclerosis
has been driven by various clinic-based and population-based studies. A frontotemporal …
has been driven by various clinic-based and population-based studies. A frontotemporal …