BiochemistrybioRxiv
Ubiquitin and ATP-dependent unfoldase activity of P97/VCP·NPLOC4·UFD1L1 is enhanced by a mutation that causes multisystem proteinopathy
E. Blythe, K. Olson, V. Chau, R. Deshaies
The paper demonstrates that wild-type p97 unfolds proteins via NPLOC4-UFD1L, ATP hydrolysis, and ubiquitination, and shows a p97 mutation enhances this activity, potentially contributing to IBMPFD pathogenesis.
DOI 10.1073/pnas.1706205114