Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium
The Dementia with Lewy Bodies (DLB) Consortium has refined its recommendations about
the clinical and pathologic diagnosis of DLB, updating the previous report, which has been …
the clinical and pathologic diagnosis of DLB, updating the previous report, which has been …
Decoding ALS: from genes to mechanism
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 …
disease. A plethora of genetic factors have been identified that drive the degeneration of …
Toxic proteins in neurodegenerative disease
JP Taylor, J Hardy, KH Fischbeck - science, 2002 - science.org
A broad range of neurodegenerative disorders is characterized by neuronal damage that
may be caused by toxic, aggregation-prone proteins. As genes are identified for these …
may be caused by toxic, aggregation-prone proteins. As genes are identified for these …
[PDF][PDF] Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillization
A Molliex, J Temirov, J Lee, M Coughlin, AP Kanagaraj… - Cell, 2015 - cell.com
Stress granules are membrane-less organelles composed of RNA-binding proteins (RBPs)
and RNA. Functional impairment of stress granules has been implicated in amyotrophic …
and RNA. Functional impairment of stress granules has been implicated in amyotrophic …
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS
HJ Kim, NC Kim, YD Wang, EA Scarborough, J Moore… - Nature, 2013 - nature.com
Algorithms designed to identify canonical yeast prions predict that around 250 human
proteins, including several RNA-binding proteins associated with neurodegenerative …
proteins, including several RNA-binding proteins associated with neurodegenerative …
[PDF][PDF] Exome sequencing reveals VCP mutations as a cause of familial ALS
JO Johnson, J Mandrioli, M Benatar, Y Abramzon… - Neuron, 2010 - cell.com
Using exome sequencing, we identified a p. R191Q amino acid change in the valosin-
containing protein (VCP) gene in an Italian family with autosomal dominantly inherited …
containing protein (VCP) gene in an Italian family with autosomal dominantly inherited …
[PDF][PDF] G3BP1 is a tunable switch that triggers phase separation to assemble stress granules
The mechanisms underlying ribonucleoprotein (RNP) granule assembly, including the basis
for establishing and maintaining RNP granules with distinct composition, are unknown. One …
for establishing and maintaining RNP granules with distinct composition, are unknown. One …
HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS
A prominent feature of late-onset neurodegenerative diseases is accumulation of misfolded
protein in vulnerable neurons. When levels of misfolded protein overwhelm degradative …
protein in vulnerable neurons. When levels of misfolded protein overwhelm degradative …
Authenticity and sincerity in tourism
JP Taylor - Annals of tourism research, 2001 - Elsevier
The paper explores the concept of authenticity with regard to the presentation of Maori in
New Zealand. It is argued that the creation of authenticity is important to tourism as a …
New Zealand. It is argued that the creation of authenticity is important to tourism as a …
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
Abstract The GGGGCC (G4C2) repeat expansion in a noncoding region of C9orf72 is the
most common cause of sporadic and familial forms of amyotrophic lateral sclerosis and …
most common cause of sporadic and familial forms of amyotrophic lateral sclerosis and …