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View article: Drug-Induced Reversible Lysosomal Changes Tracked in Live Cells by Holo-Tomographic Flow Cytometry
Drug-Induced Reversible Lysosomal Changes Tracked in Live Cells by Holo-Tomographic Flow Cytometry Open
Lysosomal storage diseases (LSDs) are genetic disorders caused by enzyme deficiencies that lead to lysosomal dysfunction and progressive cell damage. Accurate visualization and quantification of lysosomal morphology and subcellular localiz…
View article: Quantitative profiling of lysosomal accumulation through label-free biomarkers via High-Content Holo-Tomographic Flow Cytometry
Quantitative profiling of lysosomal accumulation through label-free biomarkers via High-Content Holo-Tomographic Flow Cytometry Open
Lysosomal storage diseases (LSDs) are genetic disorders caused by enzyme deficiencies that lead to lysosomal dysfunction and progressive cell damage. Accurate visualization and quantification of lysosomes are essential for understanding di…
View article: Sestrin2 drives ER-phagy in response to protein misfolding
Sestrin2 drives ER-phagy in response to protein misfolding Open
View article: TFEB controls syncytiotrophoblast formation and hormone production in placenta
TFEB controls syncytiotrophoblast formation and hormone production in placenta Open
View article: PAK6 promotes neuronal autophagy by regulating TFEB nuclear translocation.
PAK6 promotes neuronal autophagy by regulating TFEB nuclear translocation. Open
Autophagy is a highly conserved homeostatic process essential for the bulk degradation of cytoplasmic components and aggregated proteins. Multiple evidence indicates that impairment of (macro)autophagy leads to neurodegeneration, such as P…
View article: <scp>TPC2</scp> rescues lysosomal storage in mucolipidosis type <scp>IV</scp> , <scp>Niemann–Pick</scp> type <scp>C1,</scp> and Batten disease
<span>TPC2</span> rescues lysosomal storage in mucolipidosis type <span>IV</span> , <span>Niemann–Pick</span> type <span>C1,</span> and Batten disease Open
View article: Current methods to analyze lysosome morphology, positioning, motility and function
Current methods to analyze lysosome morphology, positioning, motility and function Open
Since the discovery of lysosomes more than 70 years ago, much has been learned about the functions of these organelles. Lysosomes were regarded as exclusively degradative organelles, but more recent research has shown that they play essent…
View article: Fluoxetine ameliorates mucopolysaccharidosis type IIIA
Fluoxetine ameliorates mucopolysaccharidosis type IIIA Open
Mucopolysaccharidosis type IIIA (MPS-IIIA) is an autosomal recessive disorder caused by mutations in SGSH involved in the degradation of heparan sulfate. MPS-IIIA presents severe neurological symptoms such as progressive developmental dela…
View article: Drug Repurposing in Rare Diseases: An Integrative Study of Drug Screening and Transcriptomic Analysis in Nephropathic Cystinosis
Drug Repurposing in Rare Diseases: An Integrative Study of Drug Screening and Transcriptomic Analysis in Nephropathic Cystinosis Open
Diagnosis and cure for rare diseases represent a great challenge for the scientific community who often comes up against the complexity and heterogeneity of clinical picture associated to a high cost and time-consuming drug development pro…
View article: Repurposing of tamoxifen ameliorates CLN3 and CLN7 disease phenotype
Repurposing of tamoxifen ameliorates CLN3 and CLN7 disease phenotype Open
View article: Human iPSC-Derived 2D and 3D Platforms for Rapidly Assessing Developmental, Functional, and Terminal Toxicities in Neural Cells
Human iPSC-Derived 2D and 3D Platforms for Rapidly Assessing Developmental, Functional, and Terminal Toxicities in Neural Cells Open
With increasing global health threats has come an urgent need to rapidly develop and deploy safe and effective therapies. A common practice to fast track clinical adoption of compounds for new indications is to repurpose already approved t…
View article: TRPML1 links lysosomal calcium to autophagosome biogenesis through the activation of the CaMKKβ/VPS34 pathway
TRPML1 links lysosomal calcium to autophagosome biogenesis through the activation of the CaMKKβ/VPS34 pathway Open
View article: Multi-center screening of the Pathogen Box collection for schistosomiasis drug discovery
Multi-center screening of the Pathogen Box collection for schistosomiasis drug discovery Open
View article: MOESM1 of Multi-center screening of the Pathogen Box collection for schistosomiasis drug discovery
MOESM1 of Multi-center screening of the Pathogen Box collection for schistosomiasis drug discovery Open
Additional file 1: Table S1. Descriptors and associated severity scores applied by UCSD. Table S2. Concordance between the 3 groups for active and inactive compounds. Table S3. Data transformation. Table S4. Unique hits per group. Table S5…
View article: Author Correction: Blocking Zika virus vertical transmission
Author Correction: Blocking Zika virus vertical transmission Open
A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has not been fixed in the paper.
View article: High-Throughput Screening Identifies Kinase Inhibitors That Increase Dual Adeno-Associated Viral Vector Transduction<i>In Vitro</i>and in Mouse Retina
High-Throughput Screening Identifies Kinase Inhibitors That Increase Dual Adeno-Associated Viral Vector Transduction<i>In Vitro</i>and in Mouse Retina Open
Retinal gene therapy based on adeno-associated viral (AAV) vectors is safe and efficient in humans. The low intrinsic DNA transfer capacity of AAV has been expanded by dual vectors where a large expression cassette is split in two halves i…
View article: Blocking Zika virus vertical transmission
Blocking Zika virus vertical transmission Open
The outbreak of the Zika virus (ZIKV) has been associated with increased incidence of congenital malformations. Although recent efforts have focused on vaccine development, treatments for infected individuals are needed urgently. Sofosbuvi…
View article: Activation of the transcription factor EB rescues lysosomal abnormalities in cystinotic kidney cells
Activation of the transcription factor EB rescues lysosomal abnormalities in cystinotic kidney cells Open
View article: Identification of p38 MAPK and JNK as new targets for correction of Wilson disease‐causing ATP7B mutants
Identification of p38 MAPK and JNK as new targets for correction of Wilson disease‐causing ATP7B mutants Open
Wilson disease (WD) is an autosomal recessive disorder that is caused by the toxic accumulation of copper (Cu) in the liver. The ATP7B gene, which is mutated in WD, encodes a multitransmembrane domain adenosine triphosphatase that traffics…
View article: SPR analysis of <i>H. pylori</i> RF-LPS binding to TFF1 C-terminus.
SPR analysis of <i>H. pylori</i> RF-LPS binding to TFF1 C-terminus. Open
The biotinylated synthetic peptides are composed of the last 16 aa of native TFF1. Panel A, B, C show the analysis of peptide monomers, dimers and scrambled monomers respectively challenged with H. pylori RF-LPS. Panels D, E, F show…
View article: A reverse-engineering approach to dissect post-translational modulators of transcription factor’s activity from transcriptional data
A reverse-engineering approach to dissect post-translational modulators of transcription factor’s activity from transcriptional data Open