Effect of the AMB and EGT treatments on mitochondrial membrane potential (Δψ).
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posted on 2021-10-25, 17:30 authored by Margarita M. Ivanova, Julia Dao, Neil Kasaci, Benjamin Adewale, Shaista Nazari, Lauren Noll, Jacqueline Fikry, Armaghan Hafez Sanati, Ozlem Goker-AlpanPBMC derived from GD3 patient were treated with AMB and EGT for 5 days. The JC-1 assay was measured Δψ using Flow cytometry. A. Histogram of JC-1 red (left) and green (right) fluorescence intensity of mitochondrial polarization. AMB and EGT induce JC-1 aggregations, as is shown by a shift towards increasing red fluorescent signal. B. Scatterplot shows the identification of cells with polarized (light green) and depolarized (red) mitochondria in GD3 samples. Double-stained cells are present in areas P1, P2, and P4. Cells with preferential green staining are found in area P3.
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late endosomal compartmentsglucocerebrosidase gene (<catalyzes gc biosynthesisaccumulated gc leadssevere systemic involvementdifferent molecular mechanismsbetter tissue distributionmutated gcase activityenhance gcase activitysubstrate reduction therapyproper lysosomal traffickingprogressive bone diseasemostly missense mutationsgd types 2enzyme replacement therapydiv >< pcentral nervous systemenhanced gcase activitymitochondrial membrane potentialgcase activitynervous systembone involvementgc ).mitochondrial functiongaucher diseaseequal distributiondifferent organssuggesting enhancementsuccessfully treatssubsequent accumulationsmall moleculesresults demonstraterecombinant enzymepharmacologic chaperonepartially recoversneuronopathic formsminimal effectlysosomal pathwaylysosomal dysfunctioninhibiting udpindividualized responsehematological parametershematologic manifestationshealthy controlsgba direct impactcell linesbrain barrierbiochemical responseamb enhanced
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