Figure 3.

A model for ATXN3 in the editing of Ub chains on nuclear targets. The model combines the recent work of Scaglione et al. (2011) with earlier studies showing that ATXN3, a deubiquitinating enzyme, has a role in regulating gene expression. Like the proposed role of ATXN3 in the Ube2W/CHIP ubiquitination cycle of misfolded proteins, it is postulated that ATXN3 also regulates a ubiquitination cycle involved in assembly/disassembly of transcription complexes in the nucleus. ↑[Q]n indicates an increased interaction of expanded polyQ ATXN3 with a yet to be identified nuclear protein involved in regulation of transcription.

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