Cytokine-Induced Cytotoxicity and Extracellular Matrix Abnormalities in Hepatocytes Derived From RAD50-Interacting Protein 1-Deficient Induced Pluripotent Stem Cells.

ABSTRACT: RAD50-interacting protein1 (RINT1) deficiency has been implicated in recurrent acute liver failure (RALF) triggered by fever or infections. RINT1, together with neuroblastoma amplified sequence and Zeste White 10 (forming the NRZ complex), localizes at the interface between the endoplasmic reticulum and Golgi apparatus, where it plays a key role in vesicular trafficking. However, the mechanisms by which RINT1 deficiency leads to RALF remain unclear. This study aimed to describe a woman with RALF harboring a homozygous missense mutation in RINT1. Induced pluripotent stem cells (iPSCs) were generated from the patient's mononuclear cells and differentiated into hepatocyte-like cells (HLCs). Upon exposure to high temperature (40°C), RINT1-deficient HLCs exhibited cellular damage characteristic

SUBMITTER: Zhang Y 

PROVIDER: S-EPMC12323568 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature