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WWC proteins–mediated compensatory mechanism restricts schwannomatosis driven by NF2 loss of function

22 Jan 2025

Abstract excerpt

NF2-related schwannomatosis, previously known as neurofibromatosis type 2, is a genetic disorder characterized by nerve tumors due to NF2 gene mutations. Mice with Nf2 deletion develop schwannomas slowly with low penetrance, hence inconvenient for preclinical studies. Here, we show that NF2, by recruiting E3 ubiquitin ligases β-TrCP1/2, promotes WWC1-3 ubiquitination and degradation. In NF2 mutated cells, WWC1-3...

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PMID: 39841844
DOI: 10.1126/sciadv.adp4765

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