α-Glycosidases are carbohydrate-digesting enzymes that catalyze the hydrolysis of α-1,4-glycopyranoside bonds from oligosaccharides and disaccharides. α-Glucosidase is an important biomarker for the diagnosis of type-II diabetes, Azoospermia and Pompe diseases. Additionally, the mutations in α-galactosidase lead to Fabry disease. Inhibitors targeting these enzymes are prescribed as anti-diabetic medications and as effective chaperones for Fabry disease. Comprehending the function - regulation of α-glycosidases requires accurate quantification methods. In this work, we highlight the design of a simple luminescent 'turn-on' assay for sensing these two α-glycosidases in a supramolecular TbCh hydrogel matrix using 1-α-glycosides as pro-sensitizers. The protocol offers a cost-effective method for selectively sensing α-glycosidases in the detection limit of the subnanomolar range. Importantly, the developed enzyme sensors functioned as a platform for rapid screening of drug molecules based on their inhibition potency. Therefore, the protocol is useful for facilitating the advancement of therapeutics and diagnostics targeting this important class of enzymes.
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http://dx.doi.org/10.1002/asia.202401091 | DOI Listing |
Int J Cardiovasc Imaging
January 2025
Michigan Medicine, University Hospital, Floor B1 Reception C 1500 E Medical Center Dr SPC 5030, Ann Arbor, MI, 48109, USA.
Anderson-Fabry disease (AFD) is a X-linked lysosomal storage disorder that can result in cardiac dysfunction including left ventricular hypertrophy (LVH) and conduction abnormalities (Frontiers in cardiovascular medicine vol. 10) [1]. The manifestations of AFD in women may be isolated to one organ and occur late in life due to the random inactivation of the X chromosome.
View Article and Find Full Text PDFKidney Med
January 2025
Division of Nephrology, Florida State University School of Medicine, Tallahassee, FL.
Artificial intelligence (AI) is increasingly used in many medical specialties. However, nephrology has lagged in adopting and incorporating machine learning techniques. Nephrology is well positioned to capitalize on the benefits of AI.
View Article and Find Full Text PDFCan J Kidney Health Dis
January 2025
Multiorgan Transplant Program, Division of Nephrology, Department of Medicine, McGill University Health Centre, Montreal, QC, Canada.
Background: Kidney failure is a prevalent condition with tendency for familial clustering in up to 27% of the affected individuals. Living kidney donor (LKD) transplantation is the optimal treatment option; however, in Canada, more than 45% of LKDs are biologically related to their recipients which subjects recipients to worse graft survival and donors to higher future risk of kidney failure. Although not fully understood, this observation could be partially explained by genetic predisposition to kidney diseases.
View Article and Find Full Text PDFOpen Heart
January 2025
Department of Internal Medicine I, Universitätsklinikum Würzburg, Würzburg, BY, Germany
Background And Aims: Hypertrophic cardiomyopathy (HCM) has various aetiologies, including genetic conditions like Fabry disease (FD), a lysosomal storage disorder. FD prevalence in high-risk HCM populations ranges from 0.3% to 11.
View Article and Find Full Text PDFClin 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.
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