Search Results - "Spratley, Samantha J."
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The mechanism of glycosphingolipid degradation revealed by a GALC-SapA complex structure
Published in Nature communications (11-01-2018)“…Sphingolipids are essential components of cellular membranes and defects in their synthesis or degradation cause severe human diseases. The efficient…”
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2
A simeprevir-inducible molecular switch for the control of cell and gene therapies
Published in Nature communications (27-11-2023)“…Chemical inducer of dimerization (CID) modules can be used effectively as molecular switches to control biological processes, and thus there is significant…”
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Protein Kinase D Regulates Cofilin Activity through p21-activated Kinase 4
Published in The Journal of biological chemistry (30-09-2011)“…Dynamic reorganization of the actin cytoskeleton at the leading edge is required for directed cell migration. Cofilin, a small actin-binding protein with…”
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Protein kinase d isoforms differentially modulate cofilin-driven directed cell migration
Published in PloS one (19-05-2014)“…Protein kinase D (PKD) enzymes regulate cofilin-driven actin reorganization and directed cell migration through both p21-activated kinase 4 (PAK4) and the…”
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Protein kinase D-mediated phosphorylation at Ser99 regulates localization of p21-activated kinase 4
Published in Biochemical journal (15-10-2013)“…PAKs (p21-activated kinases) are effectors of RhoGTPases. PAK4 contributes to regulation of cofilin at the leading edge of migrating cells through activation…”
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A Tetrameric Assembly of Saposin A: Increasing Structural Diversity in Lipid Transfer Proteins
Published in Contact (01-01-2021)“…Saposins are lipid transfer proteins required for the degradation of sphingolipids in the lysosome. These small proteins bind lipids by transitioning from a…”
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New therapeutic approaches for Krabbe disease: The potential of pharmacological chaperones
Published in Journal of neuroscience research (01-11-2016)“…Missense mutations in the lysosomal hydrolase β‐galactocerebrosidase (GALC) account for at least 40% of known cases of Krabbe disease (KD). Most of these…”
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Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants
Published in Traffic (Copenhagen, Denmark) (01-08-2016)“…Krabbe disease is a devastating neurodegenerative disorder caused by defects in the lysosomal hydrolase galactocerebrosidase (GALC). Over 100 different…”
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Azasugar inhibitors as pharmacological chaperones for Krabbe disease
Published in Chemical science (Cambridge) (20-05-2015)“…Krabbe disease is a devastating neurodegenerative disorder characterized by rapid demyelination of nerve fibers. This disease is caused by defects in the…”
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Abstract 2364: Regulation of cofilin activity and tumor cell migration through protein kinase D
Published in Cancer research (Chicago, Ill.) (15-04-2011)“…Abstract During early metastatic events in cancer, reorganization of the actin cytoskeleton must occur to allow for cell invasion and migration. A key enzyme…”
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Azasugar inhibitors as pharmacological chaperones for Krabbe diseaseElectronic supplementary information (ESI) available: Details of IGF, AGF, IGL and DIL syntheses including NMR spectra. Table containing X-ray data collection and refinement statistics. Dose dependence of thermal stabilization of GALC. Controls for DSF assays. IGF- and AGF-mediated stabilization of GALC is buffer and pH dependent. See DOI: 10.1039/c5sc00754bData deposition: the atomic coordinates and structure factors have been
Published 20-04-2015“…Krabbe disease is a devastating neurodegenerative disorder characterized by rapid demyelination of nerve fibers. This disease is caused by defects in the…”
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12
Azasugar inhibitors as pharmacological chaperones for Krabbe disease† †Electronic supplementary information (ESI) available: Details of IGF, AGF, IGL and DIL syntheses including NMR spectra. Table containing X-ray data collection and refinement statistics. Dose dependence of thermal stabilization of GALC. Controls for DSF assays. IGF- and AGF-mediated stabilization of GALC is buffer and pH dependent. See DOI: 10.1039/c5sc00754b Click here for additional data file. ‡ ‡Data deposition: the atomic
Published in Chemical science (Cambridge) (23-03-2015)“…Modified azasugar molecules have been synthesized and characterized as excellent pharmacological chaperone candidates to treat the neurodegenerative disorder…”
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