Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
TDP‐43 and FUS en route from the nucleus to the cytoplasm
H Ederle, D Dormann - FEBS letters, 2017 - Wiley Online Library
Misfolded or mislocalized RNA‐binding proteins (RBP s) and, consequently, altered mRNA
processing, can cause neuronal dysfunction, eventually leading to neurodegeneration. Two …
processing, can cause neuronal dysfunction, eventually leading to neurodegeneration. Two …
[HTML][HTML] Formation and maturation of phase-separated liquid droplets by RNA-binding proteins
Eukaryotic cells possess numerous dynamic membrane-less organelles, RNP granules,
enriched in RNA and RNA-binding proteins containing disordered regions. We demonstrate …
enriched in RNA and RNA-binding proteins containing disordered regions. We demonstrate …
[HTML][HTML] Residue-by-residue view of in vitro FUS granules that bind the C-terminal domain of RNA polymerase II
Phase-separated states of proteins underlie ribonucleoprotein (RNP) granules and nuclear
RNA-binding protein assemblies that may nucleate protein inclusions associated with …
RNA-binding protein assemblies that may nucleate protein inclusions associated with …
[HTML][HTML] A solid beta-sheet structure is formed at the surface of FUS droplets during aging
Phase transitions are important to understand cell dynamics, and the maturation of liquid
droplets is relevant to neurodegenerative disorders. We combined NMR and Raman …
droplets is relevant to neurodegenerative disorders. We combined NMR and Raman …
Biochemical properties and biological functions of FET proteins
Members of the FET protein family, consisting of FUS, EWSR1, and TAF15, bind to RNA and
contribute to the control of transcription, RNA processing, and the cytoplasmic fates of …
contribute to the control of transcription, RNA processing, and the cytoplasmic fates of …
[HTML][HTML] Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis
Y Shang, EJ Huang - Brain research, 2016 - Elsevier
Recent advances in the genetics of amyotrophic lateral sclerosis (ALS) have provided key
mechanistic insights to the pathogenesis of this devastating neurodegenerative disease …
mechanistic insights to the pathogenesis of this devastating neurodegenerative disease …
Two familial ALS proteins function in prevention/repair of transcription-associated DNA damage
Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron dysfunction disease that
leads to paralysis and death. There is currently no established molecular pathogenesis …
leads to paralysis and death. There is currently no established molecular pathogenesis …
ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons
J Lenzi, R De Santis, V De Turris… - Disease models & …, 2015 - journals.biologists.com
Patient-derived induced pluripotent stem cells (iPSCs) provide an opportunity to study
human diseases mainly in those cases for which no suitable model systems are available …
human diseases mainly in those cases for which no suitable model systems are available …
[HTML][HTML] The role of post-translational modifications on prion-like aggregation and liquid-phase separation of FUS
SN Rhoads, ZT Monahan, DS Yee… - International journal of …, 2018 - mdpi.com
Subcellular mislocalization and aggregation of the human FUS protein occurs in neurons of
patients with subtypes of amyotrophic lateral sclerosis and frontotemporal dementia. FUS is …
patients with subtypes of amyotrophic lateral sclerosis and frontotemporal dementia. FUS is …