[PDF][PDF] Truncated ERG oncoproteins from TMPRSS2-ERG fusions are resistant to SPOP-mediated proteasome degradation

J An, S Ren, SJ Murphy, S Dalangood, C Chang… - Molecular cell, 2015 - cell.com
J An, S Ren, SJ Murphy, S Dalangood, C Chang, X Pang, Y Cui, L Wang, Y Pan, X Zhang…
Molecular cell, 2015cell.com
Summary SPOP mutations and TMPRSS2-ERG rearrangements occur collectively in up to
65% of human prostate cancers. Although the two events are mutually exclusive, it is unclear
whether they are functionally interrelated. Here, we demonstrate that SPOP, functioning as
an E3 ubiquitin ligase substrate-binding protein, promotes ubiquitination and proteasome
degradation of wild-type ERG by recognizing a degron motif at the N terminus of ERG.
Prostate cancer-associated SPOP mutations abrogate the SPOP-mediated degradation …
Summary
SPOP mutations and TMPRSS2-ERG rearrangements occur collectively in up to 65% of human prostate cancers. Although the two events are mutually exclusive, it is unclear whether they are functionally interrelated. Here, we demonstrate that SPOP, functioning as an E3 ubiquitin ligase substrate-binding protein, promotes ubiquitination and proteasome degradation of wild-type ERG by recognizing a degron motif at the N terminus of ERG. Prostate cancer-associated SPOP mutations abrogate the SPOP-mediated degradation function on the ERG oncoprotein. Conversely, the majority of TMPRSS2-ERG fusions encode N-terminal-truncated ERG proteins that are resistant to the SPOP-mediated degradation because of degron impairment. Our findings reveal degradation resistance as a previously uncharacterized mechanism that contributes to elevation of truncated ERG proteins in prostate cancer. They also suggest that overcoming ERG resistance to SPOP-mediated degradation represents a viable strategy for treatment of prostate cancers expressing either mutated SPOP or truncated ERG.
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