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RIT1 oncoproteins escape LZTR1-mediated proteolysis

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Science  15 Mar 2019:
Vol. 363, Issue 6432, pp. 1226-1230
DOI: 10.1126/science.aav1444

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Defective degradation as disease mechanism

Ubiquitination often targets proteins for destruction. Castel et al. describe a mechanism by which mutations in the small guanine triphosphatase RIT1 may act to cause certain developmental disorders and cancers. They detected a protein, LZTR1, that interacted with wild-type RIT1 but not with oncogenic mutant forms of RIT1. LZTR1 acts as a substrate-specific adaptor for a ubiquitin ligase. Altered forms of RIT1 that are not subject to ubiquitin-mediated degradation thus accumulate. Because RIT1 functions in growth factor signaling and excessive signaling, these findings may explain the malignancies associated with RIT1 mutations.

Science, this issue p. 1226