Liquid–liquid phase separation and its mechanistic role in pathological protein aggregation
WM Babinchak, WK Surewicz - Journal of molecular biology, 2020 - Elsevier
Liquid–liquid phase separation (LLPS) of proteins underlies the formation of membrane-less
organelles. While it has been recognized for some time that these organelles are of key …
organelles. While it has been recognized for some time that these organelles are of key …
Frontotemporal lobar degeneration
Frontotemporal lobar degeneration (FTLD) is one of the most common causes of early-onset
dementia and presents with early social–emotional–behavioural and/or language changes …
dementia and presents with early social–emotional–behavioural and/or language changes …
Nuclear speckles: molecular organization, biological function and role in disease
L Galganski, MO Urbanek… - Nucleic acids …, 2017 - academic.oup.com
The nucleoplasm is not homogenous; it consists of many types of nuclear bodies, also
known as nuclear domains or nuclear subcompartments. These self-organizing structures …
known as nuclear domains or nuclear subcompartments. These self-organizing structures …
[PDF][PDF] ALS/FTD mutation-induced phase transition of FUS liquid droplets and reversible hydrogels into irreversible hydrogels impairs RNP granule function
T Murakami, S Qamar, JQ Lin, GSK Schierle, E Rees… - Neuron, 2015 - cell.com
The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS
and FTD remain controversial. We propose a model in which low-complexity (LC) domains …
and FTD remain controversial. We propose a model in which low-complexity (LC) domains …
The role of liquid–liquid phase separation in aggregation of the TDP-43 low-complexity domain
WM Babinchak, R Haider, BK Dumm, P Sarkar… - Journal of Biological …, 2019 - ASBMB
Pathological aggregation of the transactive response DNA-binding protein of 43 kDa (TDP-
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …
Chronic neuropathologies of single and repetitive TBI: substrates of dementia?
Traumatic brain injury (TBI) has long been recognized to be a risk factor for dementia. This
association has, however, only recently gained widespread attention through the increased …
association has, however, only recently gained widespread attention through the increased …
Protein aggregation in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …
aggregation of ubiquitinated proteins in affected motor neurons. Recent studies have …
Cryo-EM structure of amyloid fibrils formed by the entire low complexity domain of TDP-43
Q Li, WM Babinchak, WK Surewicz - Nature communications, 2021 - nature.com
Amyotrophic lateral sclerosis and several other neurodegenerative diseases are associated
with brain deposits of amyloid-like aggregates formed by the C-terminal fragments of TDP …
with brain deposits of amyloid-like aggregates formed by the C-terminal fragments of TDP …
Progranulin, lysosomal regulation and neurodegenerative disease
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells
MF Burkhardt, FJ Martinez, S Wright, C Ramos… - Molecular and Cellular …, 2013 - Elsevier
Abstract Development of therapeutics for genetically complex neurodegenerative diseases
such as sporadic amyotrophic lateral sclerosis (ALS) has largely been hampered by lack of …
such as sporadic amyotrophic lateral sclerosis (ALS) has largely been hampered by lack of …