Sabine Schmitt

TL;DR: It is demonstrated that selenolate-based catalysis of the essential mammalian selenoprotein GPX4 is unexpectedly dispensable for normal embryogenesis and the survival of a specific type of interneurons emerges to exclusively depend on selenocysteine-containing GPX 4, thereby preventing fatal epileptic seizures.

TL;DR: Haploid genetic screening of cells under different types of mitochondrial perturbation shows that a pathway involving OMA1, DELE1 and the eIF2α kinase HRI communicates mitochondrial stress to the cytosol to trigger the integrated stress response.

TL;DR: It is suggested that the mitochondrion constitutes a pivotal target of copper in Wilson disease, and copper-chelating agents reversed mitochondrial accumulation of copper, as well as signs of intra-mitochondrial membrane crosslinking, thereby preserving the functional and structural integrity of mitochondria.

TL;DR: It is reported that human MDSCs were characterized by strongly reduced metabolism and conferred this compromised metabolic state to CD8 + T cells, thereby paralyzing their effector functions and identifying the dicarbonyl methylglyoxal as a marker metabolite for MDSC that mediates T cell paralysis and can serve as a target to improve cancer immune therapy.

TL;DR: It is demonstrated that ATP7B-deficient rats recapitulate WD-associated phenotypes, including hepatic copper accumulation, liver damage, and mitochondrial impairment, and methanobactin treatment prevented hepatocyte death, subsequent liver failure, and death in the rodent model, suggesting that MB has potential as a therapeutic agent for the treatment of acute Wilson disease.