Effects of anserine on oxidative stress and on cell barrier integrity in methylmalonic aciduria.

ABSTRACT: Kidney damage in individuals with methylmalonyl-CoA mutase deficiency (mut⁰) results from metabolic and oxidative stress, and disrupted mitochondrial homeostasis. Anserine, known for its antioxidant properties and protective effect on cell barrier integrity of endothelial and epithelial kidney and vascular cells, may offer therapeutic potential for chronic disorders. This study explored anserine's effects on immortalized kidney tubular epithelial cells (iKTEC) from mut⁰ patients. Compared to healthy controls, iKTEC from mut⁰ patients showed reduced cell viability, antioxidant capacity, oxygen consumption, and ATP production. Expression of the tight junction scaffolding protein zonula occludens-1 (ZO-1) was increased in mut⁰ cells compared to cont

SUBMITTER: Kopfer F 

PROVIDER: S-EPMC12464228 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature