Results 81 to 90 of about 14,329 (173)
Proteostasis of organelles in aging and disease
The FEBS Journal, Volume 293, Issue 18, Page 5600-5626, September 2026.Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi +5 more
wiley +1 more source
Advanced Science, Volume 13, Issue 47, 24 August 2026.STAU2 undergoes phase separation to form dynamic condensates that package target mRNAs and deliver them to the distal ends of growing neuronal dendrites. STAU2 condensates stabilize embedded mRNAs and repress their translation. Synaptic activity bidirectionally remodels STAU2 condensates, coordinating local translation of STAU2‐associated mRNAs ...
Shijing Huang +8 more
wiley +1 more source
Immunohistochemical co-localisation of FMRP, GW182 and G3BP in human fetal ovaries at 15 wga.
, 2016 A. Yellow signal indicates co-expression of G3BP and GW182. Cyan signal indicates co-expression of GW182 and FMRP. Magenta signal indicates co-expression of G3BP and FMRP. White signal indicates co-expression of G3BP, GW182 and FMRP. B.
Panagiotis Filis (3190431) +6 more
core +1 more source
MBNL1-binding sites analysis on FMRP target RNAs.
, 2023 Fragile X syndrome (FXS) is a neurodevelopmental disorder that is often modeled in Fmr1 knockout mice where the RNA-binding protein FMRP is absent. Here, we show that in Fmr1-deficient mice, RNA mis-splicing occurs in several brain regions and peripheral
Suna Jung (17550046) +3 more
core +1 more source
FMRP Links Optimal Codons to mRNA stability in Neurons [preprint] [PDF]
, 2020 Fragile X syndrome (FXS) is caused by inactivation of the FMR1 gene and loss of encoded FMRP, an RNA binding protein that represses translation of some of its target transcripts. Here we use ribosome profiling and RNA-seq to investigate the dysregulation
Richter, Joel D. +4 more
core +1 more source
Gene reactivation and FMRP production.
, 2013 (A) The effect of HDAC and DNA methylation inhibitors on FMR1 gene expression in FXS cells. Lymphoblastoid cells from an unaffected (GM06895) and affected individual (GM03200B) were treated with 10 µM 5-aza-dC for 72 hr, or with 700 µM splitomicin (SPT ...
Karen Usdin (88334) +2 more
core +1 more source
ISO6 FMRP is cleaved by calpain in isolated Cajal bodies.
, 2013 (A) The detected FMRP associated with the Cajal bodies has an apparent molecular weight lower than expected for ISO6 FMRP. Immunoblot analyses of FMRP present in Cajal bodies using different antibodies to FMRP.
Paul De Koninck (151158) +6 more
core +1 more source
Partial FMRP expression is sufficient to normalize neuronal hyperactivity in Fragile X neurons
, 2020 © 2019 The Authors. European Journal of Neuroscience published by Federation of European Neuroscience Societies and John Wiley & Sons Ltd. Fragile X syndrome (FXS) is the most common genetic form of intellectual disability caused by a CGG repeat ...
Carrie Ng +21 more
core +1 more source
PLoS Genetics, 2010 Fragile X syndrome (FXS), the most common form of inherited mental retardation, is caused by the loss of functional fragile X mental retardation protein (FMRP). FMRP is an RNA-binding protein that can regulate the translation of specific mRNAs.
Yuping Luo +13 more
doaj +1 more source
FMRP S499 is phosphorylated independent of mTORC1-S6K1 activity.
PLoS ONE, 2014 Hyperactive mammalian target of rapamycin (mTOR) is associated with cognitive deficits in several neurological disorders including tuberous sclerosis complex (TSC). The phosphorylation of the mRNA-binding protein FMRP reportedly depends on mTOR complex 1
Christopher M Bartley +2 more
doaj +1 more source