Targeting oxidative stress-induced lipid peroxidation enhances podocyte function in cystinosis.

ABSTRACT:

Background

Cystinosis is a rare, incurable lysosomal storage disease caused by mutations in the CTNS gene encoding the cystine transporter cystinosin, which leads to lysosomal cystine accumulation in all cells of the body. Patients with cystinosis display signs of podocyte damage characterized by extensive loss of podocytes into the urine at early disease stages, glomerular proteinuria, and the development of focal segmental glomerulosclerosis (FSGS) lesions. Although standard treatment with cysteamine decreases cellular cystine levels, it neither reverses glomerular injury nor prevents the loss of podocytes. Thus, pathogenic mechanisms other than cystine accumulation are involved in podocyte dysfunction in cystinosis.

Methods

We used immortalized patient-derived cystinosis,

SUBMITTER: Berlingerio SP 

PROVIDER: S-EPMC11844038 | biostudies-literature | 2025 Feb

REPOSITORIES: biostudies-literature