We identified a systemic, progressive loss of protein S‐glutathionylation—detected by nonreducing western blotting—alongside dysregulation of glutathione‐cycle enzymes in both neuronal and peripheral tissues of Taiwanese SMA mice. These alterations were partially rescued by SMN antisense oligonucleotide therapy, revealing persistent redox imbalance as ...
Sofia Vrettou, Brunhilde Wirth
wiley +1 more source
Cells of Matter—In Vitro Models for Myotonic Dystrophy
Myotonic dystrophy type 1 (DM1 also known as Steinert disease) is a multisystemic disorder mainly characterized by myotonia, progressive muscle weakness and wasting, cognitive impairments, and cardiac defects. This autosomal dominant disease is caused by
Magdalena Matloka +3 more
doaj +1 more source
Erythropoietin production by fetal mouse liver cells in response to hypoxia and adenylate cyclase stimulation [PDF]
This study was done to investigate aspects of control of extrarenal erythropoietin (Ep) production. To this end we studied the effects of three stimuli of renal Ep production in the adult, i.e.
Bauer, Christian +4 more
core +1 more source
In this study, we found that human cervical‐derived adipocytes maintain intracellular iron level by regulating the expression of iron transport‐related proteins during adrenergic stimulation. Melanotransferrin is predicted to interact with transferrin receptor 1 based on in silico analysis.
Rahaf Alrifai +9 more
wiley +1 more source
ASIC currents in cultured primate retinal amacrine/ganglion cells
Acid‐sensing ion channels (ASICs) are proton‐gated cation channels belonging to the epithelial Na + channel/degenerin superfamily. In the CNS, ASICs are involved in synaptic plasticity, learning/memory, and acidosis‐mediated injury.
Talib Saafir +4 more
doaj +1 more source
Functional characterization of infiltrating T lymphocytes in human hepatic allografts [PDF]
We have employed recently developed techniques in T-cell culturing to study the nature and function of infiltrating hepatic allograft T cells. Using the rationale that intragraft T cells are activated during cell mediated damage to the allograft, we were
Adriana Zeevi +53 more
core +1 more source
Tau acetylation at K331 has limited impact on tau pathology in vivo
We mapped tau post‐translational modifications in humanized MAPT knock‐in mice and in amyloid‐bearing double knock‐in mice. Acetylation within the repeat domain, particularly around K331, showed modest increases under amyloid pathology. To test functional relevance, we generated MAPTK331Q knock‐in mice.
Shoko Hashimoto +3 more
wiley +1 more source
Normal versus pathological cardiac fibroblast-derived extracellular matrix differentially modulates cardiosphere-derived cell paracrine properties and commitment [PDF]
Human resident cardiac progenitor cells (CPCs) isolated as cardiosphere-derived cells (CDCs) are under clinical evaluation as a therapeutic product for cardiac regenerative medicine.
Angelini, Francesco +11 more
core +3 more sources
Calpain small subunit homodimerization is robust and calcium‐independent
Calpains dimerize via penta‐EF‐hand (PEF) domains. Using single‐molecule force spectroscopy, we measured the strength and kinetics of PEF–PEF homodimer binding. The interaction is robust, shows a transient conformational step before dissociation, and remains largely insensitive to Ca2+.
Nesha May O. Andoy +4 more
wiley +1 more source
Appropriately differentiated ARPE-19 cells regain phenotype and gene expression profiles similar to those of native RPE cells. [PDF]
PurposeThe RPE cell line ARPE-19 provides a dependable and widely used alternative to native RPE. However, replication of the native RPE phenotype becomes more difficult because these cells lose their specialized phenotype after multiple passages ...
Duncan, Todd +8 more
core +2 more sources

