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Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to
pathological inclusions in ALS?
#MMPMID26557057
Aulas A
; Vande Velde C
Front Cell Neurosci
2015[]; 9
(?): 423
PMID26557057
show ga
Stress granules (SGs) are RNA-containing cytoplasmic foci formed in response to
stress exposure. Since their discovery in 1999, over 120 proteins have been
described to be localized to these structures (in 154 publications). Most of
these components are RNA binding proteins (RBPs) or are involved in RNA
metabolism and translation. SGs have been linked to several pathologies including
inflammatory diseases, cancer, viral infection, and neurodegenerative diseases
such as amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). In
ALS and FTD, the majority of cases have no known etiology and exposure to
external stress is frequently proposed as a contributor to either disease
initiation or the rate of disease progression. Of note, both ALS and FTD are
characterized by pathological inclusions, where some well-known SG markers
localize with the ALS related proteins TDP-43 and FUS. We propose that TDP-43 and
FUS serve as an interface between genetic susceptibility and environmental stress
exposure in disease pathogenesis. Here, we will discuss the role of TDP-43 and
FUS in SG dynamics and how disease-linked mutations affect this process.
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