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Peroxynitrite supports a metabolic reprogramming in merlin-deficient Schwann cells and promotes cell survival.


ABSTRACT: Neurofibromatosis type 2 (NF2) is an autosomal-dominant disorder characterized by the development of bilateral vestibular schwannomas. The NF2 gene encodes the tumor suppressor merlin, and loss of merlin activity promotes tumorigenesis and causes NF2. Cellular redox signaling has been implicated in different stages of tumor development. Among reactive nitrogen species, peroxynitrite is the most powerful oxidant produced by cells. We recently showed that peroxynitrite-mediated tyrosine nitration down-regulates mitochondrial metabolism in tumor cells. However, whether peroxynitrite supports a metabolic shift that could be exploited for therapeutic development is unknown. Here, we show that vestibular schwannomas from NF2 patients and human, merlin-deficient (MD) Schwann cells have hig

SUBMITTER: Pestoni JC 

PROVIDER: S-EPMC6663865 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature