Nuclear pore complexes in development and tissue homeostasis
V Guglielmi, S Sakuma, MA D'Angelo - Development, 2020 - journals.biologists.com
Nuclear pore complexes are multiprotein channels that span the nuclear envelope, which
connects the nucleus to the cytoplasm. In addition to their main role in the regulation of …
connects the nucleus to the cytoplasm. In addition to their main role in the regulation of …
C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy
CN Cook, Y Wu, HM Odeh, TF Gendron… - Science translational …, 2020 - science.org
TAR DNA-binding protein 43 (TDP-43) inclusions are a pathological hallmark of
frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), including cases …
frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS), including cases …
[HTML][HTML] G4C2 repeat RNA initiates a POM121-mediated reduction in specific nucleoporins in C9orf72 ALS/FTD
Through mechanisms that remain poorly defined, defects in nucleocytoplasmic transport and
accumulations of specific nuclear-pore-complex-associated proteins have been reported in …
accumulations of specific nuclear-pore-complex-associated proteins have been reported in …
Arginine-rich dipeptide-repeat proteins as phase disruptors in C9-ALS/FTD
A hexanucleotide repeat expansion GGGGCC (G4C2) within chromosome 9 open reading
frame 72 (C9orf72) is the most common genetic cause of amyotrophic lateral sclerosis and …
frame 72 (C9orf72) is the most common genetic cause of amyotrophic lateral sclerosis and …
[HTML][HTML] Nuclear import receptors directly bind to arginine-rich dipeptide repeat proteins and suppress their pathological interactions
S Hutten, S Usluer, B Bourgeois, F Simonetti, HM Odeh… - Cell reports, 2020 - cell.com
Nuclear import receptors, also called importins, mediate nuclear import of proteins and
chaperone aggregation-prone cargoes (eg, neurodegeneration-linked RNA-binding …
chaperone aggregation-prone cargoes (eg, neurodegeneration-linked RNA-binding …
Arginine forks are a widespread motif to recognize phosphate backbones and guanine nucleobases in the RNA major groove
RNA recognition by proteins is central to biology. Here we demonstrate the existence of a
recurrent structural motif, the “arginine fork”, that codifies arginine readout of cognate …
recurrent structural motif, the “arginine fork”, that codifies arginine readout of cognate …
[HTML][HTML] Nuclear periphery takes center stage: the role of nuclear pore complexes in cell identity and aging
UH Cho, MW Hetzer - Neuron, 2020 - cell.com
In recent years, the nuclear pore complex (NPC) has emerged as a key player in genome
regulation and cellular homeostasis. New discoveries have revealed that the NPC has …
regulation and cellular homeostasis. New discoveries have revealed that the NPC has …
[HTML][HTML] Phase separation of toxic dipeptide repeat proteins related to C9orf72 ALS/FTD
The expansion mutation in the C9orf72 gene is the most common known genetic cause for
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This mutation can …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). This mutation can …
[HTML][HTML] C9orf72-associated arginine-rich dipeptide repeat proteins reduce the number of Golgi outposts and dendritic branches in Drosophila neurons
Altered dendritic morphology is frequently observed in various neurological disorders
including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the …
including amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), but the …
[HTML][HTML] The sense of targeting nonsense-mediated decay in C9-ALS/FTD
Defective nucleocytoplasmic transport contributes to C9-ALS/FTD, but an inventory of
proteins that become redistributed has remained elusive. In this issue of Neuron, Ortega et …
proteins that become redistributed has remained elusive. In this issue of Neuron, Ortega et …