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Chemical imaging unveils mitochondria as the major site of medicinal biguanide accumulation within cells.
Heliyon
July 2024
School of Life Sciences, Westlake University, Hangzhou, Zhejiang, China.
Metformin (MET), a commonly prescribed medication for managing type 2 diabetes, has demonstrated various beneficial effects beyond its primary anti-diabetic efficacy. However, the mechanism underlying MET activity and its distribution within organelles remain largely unknown. In this study, we integrate multiple technologies, including chemical labeling, immunostaining, and high-resolution microscopy imaging, to visualize the accumulation of MET in organelles of cultured cells.
View Article and Find Full Text PDFPathogenic variants of GDAP1 cause Charcot-Marie-Tooth disease (CMT), an inherited neuropathy characterized by axonal degeneration. GDAP1, an atypical glutathione S-transferase, localizes to the outer mitochondrial membrane (OMM), regulating this organelle's dynamics, transport, and membrane contact sites (MCSs). It has been proposed that GDAP1 functions as a cellular redox sensor.
View Article and Find Full Text PDFLentivirus-mediated gene therapy for Fabry disease: 5-year End-of-Study results from the Canadian FACTs trial.
Clin Transl Med
January 2025
Department of Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
Background: Fabry disease is an X-linked lysosomal storage disorder due to a deficiency of α-galactosidase A (α-gal A) activity. Our goal was to correct the enzyme deficiency in Fabry patients by transferring the cDNA for α-gal A into their CD34+ hematopoietic stem/progenitor cells (HSPCs). Overexpression of α-gal A leads to secretion of the hydrolase; which can be taken up and used by uncorrected bystander cells.
View Article and Find Full Text PDFThe multiple facets of Rab proteins modulating the cellular distribution of cholesterol from the late endosomal compartment.
Biochim Biophys Acta Mol Cell Res
January 2025
School of Pharmacy, Faculty of Medicine and Health, University of Sydney, Sydney, NSW 2006, Australia. Electronic address:
Cholesterol is an essential lipid that ensures the functional integrity of mammalian cells. Most cells acquire cholesterol via endocytosis of low-density lipoproteins (LDL). Upon reaching late endosomes/lysosomes (LE/Lys), incoming ligands, including LDL-derived cholesterol, are distributed to other organelles.
View Article and Find Full Text PDFBiocompatible 2D Material Inks Enabled by Supramolecular Chemistry: From Synthesis to Applications.
Acc Chem Res
January 2025
Department of Chemistry, The University of Manchester, Manchester M13 9PL, United Kingdom.
ConspectusThe emergence of two-dimensional (2D) materials, such as graphene, transition-metal dichalcogenides (TMDs), and hexagonal boron nitride (h-BN), has sparked significant interest due to their unique physicochemical, optical, electrical, and mechanical properties. Furthermore, their atomically thin nature enables mechanical flexibility, high sensitivity, and simple integration onto flexible substrates, such as paper and plastic.The surface chemistry of a nanomaterial determines many of its properties, such as its chemical and catalytic activity.
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