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Substrate Profiling of RNF216 Uncovers a Translation-Linked OTUD4 Regulatory Axis

2026-08-30

Abstract excerpt

Mutations in the E3 Ubiquitin (Ub) ligase RNF216 cause Gordon Holmes syndrome (GHS), a neurodegenerative disorder accompanied by neuroendocrine disruption. We developed an orthogonal ubiquitin transfer (OUT) platform to capture RNF216 substrates in neuronal cells and identified OTUD4, a deubiquitinating enzyme (DUB) mutated in GHS, and FMRP, a neuronal-enriched translational repressor. RNF216 predominantly synthes...

Identifiers and source

Literature Corpus work
6c7bc193-46b3-52b5-8005-f46275eae8df
DOI
10.64898/2026.08.26.747332
Open publication

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Substrate Profiling of RNF216 Uncovers a Translation-Linked OTUD4 Regulatory AxisDOI 10.64898/2026.08.26.747332
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