UBR1 and Johanson-Blizzard syndrome: A proteasome inhibitor, MG132 has also been shown to increase the level of Ubr1, and this may act as a useful therapeutic strategy in pathologies caused due to reduced amount of Ubr1, like pancreatic dysfunctions and mental abnormalities, characteristics of Johanson-Blizzard syndrome (Zenker et al., 2005).