Chemical chaperones to the rescue of Alport syndrome?

Publisher: Elsevier Country of Publication: United States NLM ID: 0323470 Publication Model: Print Cited Medium: Internet ISSN: 1523-1755 (Electronic) Linking ISSN: 00852538 NLM ISO Abbreviation: Kidney Int Subsets: MEDLINE

Publication: 2016- : New York : Elsevier
Original Publication: New York, Springer-Verlag.

Nephritis, Hereditary*/genetics ; Nephritis, Hereditary*/drug therapy ; Nephritis, Hereditary*/pathology ; Nephritis, Hereditary*/metabolism ; Collagen Type IV*/genetics ; Collagen Type IV*/metabolism ; Taurochenodeoxycholic Acid*/pharmacology ; Taurochenodeoxycholic Acid*/therapeutic use ; Glomerular Basement Membrane*/pathology ; Glomerular Basement Membrane*/drug effects ; Autoantigens*/genetics ; Autoantigens*/metabolism; Animals ; Mice ; Humans ; Endoplasmic Reticulum Stress/drug effects ; Apoptosis/drug effects ; Disease Models, Animal ; Podocytes/drug effects ; Podocytes/pathology ; Podocytes/metabolism ; Mutation, Missense ; Molecular Chaperones/genetics ; Molecular Chaperones/metabolism

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.)

0 (Collagen Type IV)
516-35-8 (Taurochenodeoxycholic Acid)
0 (type IV collagen alpha3 chain)
60EUX8MN5X (ursodoxicoltaurine)
0 (Autoantigens)
0 (Molecular Chaperones)

Date Created: 20240822 Date Completed: 20240822 Latest Revision: 20240822

20240823

10.1016/j.kint.2024.07.006

39174196