Chemical chaperones to the rescue of Alport syndrome?
| Autor: | Vanacore RM; Division of Nephrology and Hypertension, Department of Medicine, Vanderbilt University Medical Center, Nashville, Tennessee, USA; Vanderbilt Center for Matrix Biology, Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address: roberto.vanacore@vanderbilt.edu. |
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| Jazyk: | angličtina |
| Zdroj: | Kidney international [Kidney Int] 2024 Sep; Vol. 106 (3), pp. 359-361. |
| DOI: | 10.1016/j.kint.2024.07.006 |
| Abstrakt: | Alport syndrome is a hereditary kidney disease caused by collagen IV mutations that interfere with the formation and deposition of the α3α4α5 protomer into the glomerular basement membrane. In this issue, Yu et al. show that the chemical chaperone tauroursodeoxycholic acid prevented kidney structural changes and function decline in mice with a pathogenic missense Col4a3 mutation by increasing mutant α3α4α5 protomer glomerular basement membrane deposition and preventing podocyte apoptosis induced by endoplasmic reticulum stress. (Copyright © 2024 International Society of Nephrology. Published by Elsevier Inc. All rights reserved.) |
| Databáze: | MEDLINE |
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