Results 191 to 200 of about 42,118 (292)
3′UTR variants of ALS‐linked RNAs modify subcellular and cellular phenotypes
The FEBS Journal, EarlyView.Our study demonstrates that alternative 3′UTR variants of ALS‐linked transcripts modulate subcellular RNA localization and cytoskeletal architecture. NEFH 3′UTR‐Long promotes nuclear RNA clustering, while SOD1 3′UTR‐Long reduces filopodia formation. These results suggest that 3′UTRs, independent of coding sequences, can influence neuronal phenotypes ...
Melis Savasan‐Sogut +2 more
wiley +1 more source
AAVrh10 gene therapy ameliorates central and peripheral nervous system disease in canine globoid cell leukodystrophy (Krabbe disease) [PDF]
, 2018 et al,, Jiang, Xuntain, Ory, Daniel S
core +1 more source
The FEBS Journal, EarlyView.Iduronate 2‐sulfatase (IDS; purple) is expressed as a precursor protein that goes through multiple steps of maturation, modification, and trafficking to become an active lysosomal enzyme that degrades glycosaminoglycans. Our study shows that the transmembrane ubiquitin ligases RNF13 (orange) and RNF167 (pink) heterodimerize, affecting IDS intracellular
Valérie C. Cabana +4 more
wiley +1 more source
Proteostasis of organelles in aging and disease
The FEBS Journal, EarlyView.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
The FEBS Journal, EarlyView.Exercise‐related microRNAs cel‐miR‐249‐3p and cel‐miR‐77‐5p in C. elegans regulate lifespan, fitness, mitochondrial morphology and membrane potential. Although lacking direct mammalian orthologs, H2O2‐induced microRNAs mmu‐miR‐181a‐5p and mmu‐miR‐378a‐3p regulate myogenesis, autophagy, mitochondrial content and respiration in murine myoblasts ...
Qin Xia +7 more
wiley +1 more source
A new glucocerebrosidase chaperone reduces α-synuclein and glycolipid levels in iPSC-derived dopaminergic neurons from patients with gaucher disease and parkinsonism [PDF]
, 2016 et al, +3 more
core +1 more source
The FEBS Journal, EarlyView.The extracellular matrix (ECM) is a dynamic scaffold that orchestrates tissue architecture and cellular communication. A critical but underexplored interplay between proteases and cluster of differentiation molecules (CD) governs ECM turnover and directs cell fate.
David Jurnečka +3 more
wiley +1 more source
Prospective exploratory muscle biopsy, imaging, and functional assessment in patients with late-onset Pompe disease treated with alglucosidase alfa: The EMBASSY Study [PDF]
, 2016 et al,, Pestronk, Alan
core +1 more source
The FEBS Journal, EarlyView.Divalent metal transporter 1 (Dmt1) maintains iron homeostasis and lysosomal proteostasis required for physiological Notch receptor–ligand signaling. Dmt1 loss lowers iron storage capacity (ferritin), increasing intracellular Fe2+, driving ROS and lipid peroxidation, and leading to lysosomal/mitochondrial dysfunction.
Rui Zhang +5 more
wiley +1 more source
A novel transgenic mouse model of lysosomal storage disorder.
Am J Physiol Gastrointest Liver Physiol, 2016 Ortiz-Miranda S +12 more
europepmc +1 more source