Amyotrophic lateral sclerosis: proteins, proteostasis, prions, and promises
Amyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of the
motor neurons that innervate muscle, resulting in gradual paralysis and culminating in the …
motor neurons that innervate muscle, resulting in gradual paralysis and culminating in the …
Interactions of amyloid coaggregates with biomolecules and its relevance to neurodegeneration
K Murakami, K Ono - The FASEB Journal, 2022 - repository.kulib.kyoto-u.ac.jp
The aggregation of amyloidogenic proteins is a pathological hallmark of various
neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and …
neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and …
Characterization of a family of RanBP2-type zinc fingers that can recognize single-stranded RNA
CD Nguyen, RE Mansfield, W Leung, PM Vaz… - Journal of molecular …, 2011 - Elsevier
The recognition of single-stranded RNA (ssRNA) is an important aspect of gene regulation,
and a number of different classes of protein domains that recognize ssRNA in a sequence …
and a number of different classes of protein domains that recognize ssRNA in a sequence …
[HTML][HTML] Karyopherins and condensates
Several aggregation-prone RNA-binding proteins, including FUS, EWS, TAF15, hnRNP A1,
hnRNP A2, and TDP-43, are mutated in neurodegenerative diseases. The nuclear …
hnRNP A2, and TDP-43, are mutated in neurodegenerative diseases. The nuclear …
Identification and characterization of FUS/TLS as a new target of ATM
M Gardiner, R Toth, F Vandermoere… - Biochemical …, 2008 - portlandpress.com
ATM (ataxia-telangiectasia mutated), ATR (ATM-and Rad3-related) and DNA-PK (DNA-
dependent protein kinase), important regulators of genome stability, belong to the PIKK …
dependent protein kinase), important regulators of genome stability, belong to the PIKK …
Translation dysregulation in neurodegenerative diseases: a focus on ALS
RNA translation is tightly controlled in eukaryotic cells to regulate gene expression and
maintain proteome homeostasis. RNA binding proteins, translation factors, and cell …
maintain proteome homeostasis. RNA binding proteins, translation factors, and cell …
TLS inhibits RNA polymerase III transcription
AY Tan, JL Manley - Molecular and cellular biology, 2010 - Taylor & Francis
RNA transcription by all the three RNA polymerases (RNAPs) is tightly controlled, and loss
of regulation can lead to, for example, cellular transformation and cancer. While most …
of regulation can lead to, for example, cellular transformation and cancer. While most …
RNA-binding proteins in neurological diseases
HL Zhou, M Mangelsdorf, JH Liu, L Zhu… - Science China Life …, 2014 - Springer
Emerging studies support that RNA-binding proteins (RBPs) play critical roles in human
biology and pathogenesis. RBPs are essential players in RNA processing and metabolism …
biology and pathogenesis. RBPs are essential players in RNA processing and metabolism …
Aberrant stress granule dynamics and aggrephagy in ALS pathogenesis
Y Zhang, J Gu, Q Sun - Cells, 2021 - mdpi.com
Stress granules are conserved cytosolic ribonucleoprotein (RNP) compartments that
undergo dynamic assembly and disassembly by phase separation in response to stressful …
undergo dynamic assembly and disassembly by phase separation in response to stressful …
FUS/TLS forms cytoplasmic aggregates, inhibits cell growth and interacts with TDP-43 in a yeast model of amyotrophic lateral sclerosis
D Kryndushkin, RB Wickner, F Shewmaker - Protein & cell, 2011 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is a fatal disease characterized by the premature loss of
motor neurons. While the underlying cellular mechanisms of neuron degeneration are …
motor neurons. While the underlying cellular mechanisms of neuron degeneration are …