AI Article Synopsis
- - Alport syndrome is a genetic kidney disease linked to mutations in collagen IV that disrupt the formation of a key protein complex in the kidney's structure.
- - Recent research by Yu et al. demonstrates that tauroursodeoxycholic acid, a chemical chaperone, can protect against kidney damage in mice with a specific Col4a3 mutation.
- - The treatment helps by boosting the deposition of the crucial protein complex in the kidney and stopping cell death in podocytes caused by stress on the endoplasmic reticulum.
Article Abstract
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.
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| http://dx.doi.org/10.1016/j.kint.2024.07.006 | DOI Listing |
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