Protein quality control and the amyotrophic lateral sclerosis/frontotemporal dementia continuum

H Shahheydari, A Ragagnin, AK Walker… - Frontiers in molecular …, 2017 - frontiersin.org
Protein homeostasis, or proteostasis, has an important regulatory role in cellular function.
Protein quality control mechanisms, including protein folding and protein degradation …

Intrinsically disordered RGG/RG domains mediate degenerate specificity in RNA binding

BA Ozdilek, VF Thompson, NS Ahmed… - Nucleic acids …, 2017 - academic.oup.com
RGG/RG domains are the second most common RNA binding domain in the human
genome, yet their RNA-binding properties remain poorly understood. Here, we report a …

The FUS protein: Physiological functions and a role in amyotrophic lateral sclerosis

AD Efimova, RK Ovchinnikov, AY Roman, AV Maltsev… - Molecular Biology, 2017 - Springer
Certain forms of amyotrophic lateral sclerosis (ALS) are associated with an altered
compartmentalization of FUS and its aggregation in the cytoplasm of motoneurons. FUS is a …

Intrinsic disorder in proteins involved in amyotrophic lateral sclerosis

N Santamaria, M Alhothali, MH Alfonso… - Cellular and Molecular …, 2017 - Springer
Five structurally and functionally different proteins, an enzyme superoxide dismutase 1
(SOD1), a TAR-DNA binding protein-43 (TDP-43), an RNA-binding protein FUS, a cofilin …

Nucleic acid binding proteins affect the subcellular distribution of phosphorothioate antisense oligonucleotides

JK Bailey, W Shen, X Liang, ST Crooke - Nucleic acids research, 2017 - academic.oup.com
Antisense oligonucleotides (ASOs) are versatile tools that can regulate multiple steps of
RNA biogenesis in cells and living organisms. Significant improvements in delivery, potency …

Energy homeostasis and abnormal RNA metabolism in amyotrophic lateral sclerosis

YJ Liu, PY Tsai, Y Chern - Frontiers in Cellular Neuroscience, 2017 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that is clinically
characterized by progressive muscle weakness and impaired voluntary movement due to …

Six GU-rich (6GUR) FUS-binding motifs detected by normalization of CLIP-seq by Nascent-seq

J Takeda, A Masuda, K Ohno - Gene, 2017 - Elsevier
FUS, an RNA-binding protein (RBP), is mutated or abnormally regulated in
neurodegenerative disorders. FUS regulates various aspects of RNA metabolisms. FUS …

Intrinsically disordered domains, amyloids and protein liquid phases: evolving concepts and open questions

M Mompean, DV Laurents - Protein and Peptide Letters, 2017 - ingentaconnect.com
Enzymes and structural proteins dominated thinking about protein structure and function for
most of the twentieth century. In recent decades, however, we have begun to appreciate the …

Белок FUS: физиологические функции и роль в развитии бокового амиотрофического склероза

АД Ефимова, РК Овчинников, АЮ Роман… - Молекулярная …, 2017 - elibrary.ru
При некоторых формах бокового амиотрофического склероза наблюдается
нарушение компарт-ментализации белка FUS и его агрегация в цитоплазме …

Characterizing the RNA binding properties of the intrinsically disordered FUS protein and RGG/RG domains

BA Ozdilek - 2017 - search.proquest.com
Recent developments in the comprehensive identification of the RNA-binding protein (RBP)
repertoire has accelerated discovery of new RBPs. According to these studies, 20% of both …