Results 71 to 80 of about 2,687,044 (278)
Leveraging the chemical similarity between Ga3+ and Fe3+ as well as the antioxidant properties of quercetin, gallium–quercetin nanoparticles (GQNPs) were prepared to integrate iron homeostasis regulation, oxidative stress suppression, and mitochondrial protection for multi‐target ferroptosis inhibition in Parkinson's Disease.
Keyang Xu +12 more
wiley +1 more source
Condensates at synapses organize synaptic vesicles (SVs) and are crucial for efficient neurotransmitter release, yet how RNA contributes to this mesoscale architecture remains poorly understood. Here we uncover that RNA modulates synapsin–driven condensates.
Branislava Rankovic +21 more
wiley +1 more source
Mitochondrial stress markers associate with phenotypic variability in Fabry disease
Background Fabry disease (FD) exhibits marked clinical heterogeneity that cannot be fully explained by residual α-galactosidase A activity. Mitochondrial dysfunction has been reported in FD, but the role of mitochondrial stress remains unexplored ...
Lucia Lavalle +5 more
doaj +1 more source
A multifunctional EV‐based nanoplatform (Ang‐TEVs@Gel) was engineered via preconditioning, surface targeting, and ROS‐responsive hydrogel encapsulation to reprogram microglia. This system restored autophagy via miR‐664a‐3p/PIK3CA axis, cleared myelin debris, resolved neuroinflammation, and promoted axon remyelination, ultimately achieving robust motor ...
Wu Xiong +17 more
wiley +1 more source
Human iPSC‐derived Fabry cardiomyocytes exhibited broad transcriptional dysregulation, apoptosis, mitochondrial dysfunction, impaired reactive oxygen species handling, altered contractility, and abnormal calcium transient decay, potentially mediated by phospholamban hyperphosphorylation.
Malte Juchem +24 more
wiley +1 more source
Types and Genetic Evaluation of Lysosomal Storage Diseases in Kurdistan Region
Background and objectives: Lysosomal storage diseases are a set of single-gene disorders that is attributed to insufficient certain lysosomal hydrolase activity or non-enzymatic proteins vital for typical lysosomal functions.
Lana Ahmed Mohammed
doaj +1 more source
Since Christian de Duve first described the lysosome in the 1950s, it has been generally presented as a membrane-bound compartment containing acid hydrolases that enables the cell to degrade molecules without being digested by autolysis.
Ursula Matte BSc, PhD +1 more
doaj +1 more source
Lysosomal storage disorders: emerging therapeutic options require early diagnosis
Lysosomal storage disorders have been recognised as one of the major groups of genetic disorders affecting children and adults. With over 40 different disorders and a combined prevalence of up to 1:5000 births, this group of disorders is a major public ...
Hopwood, J., Meikle, P.
core +1 more source
A biomimetic doppelgänger nanosystem neutralizes extracellular inflammatory cytokines and silences intracellular pyroptosis, reprogramming pathogenic macrophages to attenuate both joint and spine degeneration. ABSTRACT Osteoarthritis (OA) and intervertebral disc degeneration (IVDD) are debilitating musculoskeletal disorders driven by shared ...
Fudong Li +9 more
wiley +1 more source
Induced pluripotent stem cell models of lysosomal storage disorders
Induced pluripotent stem cells (iPSCs) have provided new opportunities to explore the cell biology and pathophysiology of human diseases, and the lysosomal storage disorder research community has been quick to adopt this technology.
Tamanna Roshan Lal +5 more
core +2 more sources

