Global ETD Search
Search theses and dissertations gathered from participating repositories worldwide. Every result links back to the library that holds it. No account is needed.
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Showing 1 to 7 of 7 for “"TRPML1"”.
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MODULATION OF TRPML1/TFEB PATHWAY FOR THE TREATMENT OF WILSON DISEASE
… transient receptor potential mucolipin 1 (TRPML1) activates calcineurin, which dephosphorylates TFEB, promoting its nuclear translocation. In this study, we investigated the therapeutic potential of TRPML1/TFEB axis for the treatment of WD. We first assessed the effects of TFEB and TRPML1 …
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TRPML1: Role In Autophagy And Potential Target To Treat Lysosomal Storage Disorders
… Lysosomal Ca2+ release via mucolipin 1 (TRPML1) activates calcineurin, which binds and de-phosphorylates TFEB, thus promoting its nuclear translocation. Induction of autophagy and lysosomal biogenesis via TFEB required TRPML1-mediated calcineurin activation, linking lysosomal calcium …
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TRPML1 Promotes Protein Homeostasis in Melanoma Cells by Negatively Regulating MAPK and mTORC1 Signaling
… which encodes the lysosomal cation channel TRPML1, is preferentially required for the survival and proliferation of melanoma cells. Loss of MCOLN1/TRPML1 function impaired the growth of patient-derived melanomas in culture and in xenografts but did not affect the growth of human melanocytes. …
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Targeting Sequence and Function-Dependence of Subcellular Localization of Transient Receptor Potential Mucolipin Channels and Characterization of Engineered Molecule Probes For Detecting Lysosomal Calcium Release
<p>Both TRPML1 and TRPML3 are members of the mucolipin subfamily of Transient Receptor Potential (TRP) channels. They have been implicated in endolysosomal functions such as divalent cation release, luminal pH regulation, autophagy and vesicle trafficking. Interestingly, whereas TRPML1 is almost …
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Organellar Zn2+ Homeostasis and the Role of TRPML Channels in Neuronal Lysosome Physiology and Axonal Transport
… molecule sensors and GZnP3, we establish that TRPML1 and TRPML3 channels are permeable to physiological concentrations of Zn<sup>2+</sup>. Upon characterizing the location of these channels, we also provide the first direct evidence that TRPML channels can release Zn<sup>2+</sup> from …
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Developing Red Fluorescent Zn2+ Sensors and Characterizing Zn2+ Homeostasis in Mucolipidosis Type IV Disease
… loss-function mutations in the lysosomal channel TRPML1. We assess the Zn<sup>2+</sup> and Ca<sup>2+</sup> permeability and localization of 10 different TRPML1 patient mutants. Furthermore, we quantify Zn<sup>2+</sup> concentrations in the cytosol, lysosomes, and mitochondria in MLIV patient …
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Involvement of Gdap1 In The Regulation of Mtorc1 Activity In A Drosophila Mliv Model
<p>The master regulator of metabolism and growth, mechanistic target of rapamycin complex 1 (mTORC1), is responsible for maintaining metabolic homeostasis by sensing nutrient and energy levels within the cell to promote or inhibit translation and autophagy accordingly. In the childhood …