[HTML][HTML] Structure of the ubiquitin-associated domain of p62 (SQSTM1) and implications for mutations that cause Paget's disease of bone

B Ciani, R Layfield, JR Cavey, PW Sheppard… - Journal of Biological …, 2003 - ASBMB
The p62 protein (also known as SQSTM1) mediates diverse cellular functions including
control of NFκB signaling and transcriptional activation. p62 binds non-covalently to
ubiquitin and co-localizes with ubiquitylated inclusions in a number of human protein
aggregation diseases. Mutations in the gene encoding p62 cause Paget's disease of bone
(PDB), a common disorder of the elderly characterized by excessive bone resorption and
formation. All of the p62 PDB mutations identified to date cluster within the C-terminal region …