Results 41 to 50 of about 31,581 (280)

Lysosomal acid lipase deficiency in a 6-year-old child: case report

Archives of the Balkan Medical Union, 2020
Introduction. Cholesteryl ester storage disease or lysosomal acid lipase deficiency is a rare severe congenital enzyme pathology of lysosomal storage disorders.
Oleksandra SHULHAI, Anna KABAKOVA, Anna-Mariia SHULHAI   +2 more
doaj   +1 more source

Microbiome−host proteostasis crosstalk—An emerging perspective on mechanisms and interventions toward healthy longevity

FEBS Letters, EarlyView.
Proteostasis and the gut microbiota play a key role in shaping host physiology. Microbiota‐derived metabolites, vitamins, and RNA modulate host proteostasis. Findings from model systems, including C. elegans, indicate microbes can either stabilize or disrupt host proteostasis.
Abhishek Anil Dubey, Maria Ermolaeva
wiley   +1 more source

Gene therapy of lysosomal storage disorders by lentiviral vectors

, 2007
Lysosomal storage disorders (LSDs) comprise a class of inherited diseases characterized by disruption of normal lysosomal function and the consequent accumulation of incompletely degraded substrates.
Alessandra Biffi, Biffi A., Naldini L., Luigi Naldini   +3 more
core   +1 more source

Deciphering transcriptional plasticity in pancreatic ductal adenocarcinoma reveals alterations in sensory neuron innervation

Molecular Oncology, EarlyView.
Pancreatic sensory neurons innervating healthy and PDAC tissue were retrogradely labeled and profiled by single‐cell RNA sequencing. Tumor‐associated innervation showed a dominant neurofilament‐positive subtype, altered mitochondrial gene signatures, and reduced non‐peptidergic neurons.
Elena Genova, Michele Montrone, Uday Rangaswamy, Francesco Diversi, Irene Schiavo, Denise Ferrarini, Roberta Di Florio, Irene Longo, Michele Coscia, Nicola Zamboni, Giorgia Demontis, Lisa Veghini, Vincenzo Corbo, Remo Sanges, Paul Heppenstall   +14 more
wiley   +1 more source

Large‐scale bidirectional arrayed genetic screens identify OXR1 and EMC4 as modifiers of αSynuclein aggregation

FEBS Open Bio, EarlyView.
Activation of the mitochondrial protein OXR1 increases pSyn129 αSynuclein aggregation by lowering ATP levels and altering mitochondrial membrane potential, particularly in response to MSA‐derived fibrils. In contrast, ablation of the ER protein EMC4 enhances autophagic flux and lysosomal clearance, broadly reducing α‐synuclein aggregates.
Sandesh Neupane, Lea Nikolić, Lorenzo Maraio, Thomas Goiran, Nathan Karpilovsky, Stefano Sellitto, Vangelis Bouris, Jiang‐An Yin, Ronald Melki, Edward A Fon, Adriano Aguzzi, Elena De Cecco   +11 more
wiley   +1 more source

UiO‐66 metal–organic frameworks in biomedicine: From structural tunability to bioimaging, photodiagnostics, and photodynamic cancer therapy

FEBS Open Bio, EarlyView.
UiO‐66(Zr) metal–organic frameworks are chemically stable, biocompatible, and highly tunable nanomaterials. Their modular structure enables controlled drug delivery, multimodal bioimaging, and light‐activated photodynamic therapy, supporting integrated diagnostic and therapeutic (theranostic) applications in cancer and biomedical research.
Veronika Huntošová, Grigorii Rakhalskii, Miroslav Almáši   +2 more
wiley   +1 more source

The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction.

, 2012
Lysosomal storage diseases (LSDs) are a family of disorders that result from inherited gene mutations that perturb lysosomal homeostasis. LSDs mainly stem from deficiencies in lysosomal enzymes, but also in some non-enzymatic lysosomal proteins, which ...
van der Spoel, AC, Platt, Frances, Platt, FM, Barry Boland, Aarnoud C. van der Spoel, Boland, B., Platt, F. M., van der Spoel, A. C., Frances M. Platt, Boland, B   +9 more
core   +1 more source

Intrinsic Photoactive Star ZnPc–Poly(glutamate) Nanoplatforms for Multimodal Glioblastoma Therapy and Brain‐Targeted Delivery

Advanced Functional Materials, EarlyView.
An intrinsic photoactive star‐shaped zinc phtalocyanine‐poly(L‐glutamic acid) (ZnPc‐PGA) nanoplatform for multimodal glioblastoma (GBM) therapy and brain‐targeted elivery. A ZnPc‐PGA‐based multifunctional theranostic nanocarrier platform enables image‐guided, multimodal GBM therapy. ZnPc‐PGA nanocarriers support the integration of fluorescence imaging,
Amina Benaicha‐Fernández, Teodora Ranđelović, Ana Armiñán, Víctor Sebastián, Manuel Fuentes, Cristián Huck‐Iriart, Esther Masiá, Paula Carrascosa‐Marco, Pablo Juanes‐Velasco, Snežana Đorđević, Clara Bayona, Ignacio Ochoa, Maria Medel, Inmaculada Conejos‐Sánchez, María J. Vicent   +14 more
wiley   +1 more source

Functional Blood‐Brain Barrier Crossing by Biomimetic M13 Phage Vectors for Targeted Neuronal Delivery

Advanced Healthcare Materials, EarlyView.
This study investigates the M13 bacteriophage as a biomimetic nanovector capable of crossing in vitro models of the blood–brain barrier. By exploiting peculiar transcellular pathways, M13 avoids lysosomal degradation and preserves its structural integrity and functionality.
Silvia Vercellino, Lucia Pappagallo, Francesca De Chirico, Luca Boselli, Hazem Ahmed, Luca Spagnoletti, Giovanni Pittaluga, Paolo Emidio Costantini, Matteo Calvaresi, Pier Paolo Pompa, Alberto Danielli, Fabio Benfenati, Valentina Castagnola   +12 more
wiley   +1 more source

Super‐Resolution Ultrasound Based Cell Tracking With Polymeric Nanobubbles

Advanced Materials, EarlyView.
This study presents a super‐resolution ultrasound platform for tracking cells in vivo. Biocompatible polymeric nanobubbles are used as highly echogenic intracellular labels. Following the injection of cells and microbubbles, ultrasound localization microscopy (ULM) can dynamically match the microvascular architecture and individual cell trajectories ...
Junlin Chen, Xiaoyu Wang, Jilin Fan, Bi Wang, Hanghang Fang, Yurui Wang, Hao Cui, Mohammad Roufarshbaf, Ekaterina Savina, Alexandra Valeske, Quim Peña, Yang Shi, Andreas Herrmann, Twan Lammers, Mathias Hornef, Roman Barmin, Thomas Lisson, Georg Schmitz, Anne Rix, Fabian Kiessling   +19 more
wiley   +1 more source