Background: 20 read-through mutations that produce C-terminally extended proteins are related to human hereditary disorders. Results: The C-terminal extended proteins of mouse cFLIP-L (cellular FLICE-like apoptosisinhibitory protein) and human PNPO (pyridoxamine 5-phosphate oxidase) and HSD3B2 (3-hydroxysteroid dehydrogenase type II) are ubiquitylated and degraded, involving an E3 ligase, TRIM21, for cFLIP-L and PNPO degradation. Conclusion: Read-through mutant cFLIP-L, PNPO, and HSD3B2 are degraded by the ubiquitin-proteasome system. Significance: Degradation of read-through mutant proteins may cause hereditary disorders.
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